ArticleLiterature Review

Relapsing polychondritis: a clinical review for rheumatologists

November 2017
British Journal of Rheumatology 57(9)

DOI:10.1093/rheumatology/kex406

Authors:

Jack Kingdon

King's College London

Joseph Roscamp

Sheffield Teaching Hospitals NHS Foundation Trust

Shirish Sangle

Guy's and St Thomas' NHS Foundation Trust

David D'Cruz

King's College London

Relapsing polychondritis (RPC) is a rare autoimmune rheumatic disorder that is traditionally classified as a systemic vasculitis. It is characterized by inflammation of cartilage, and typical presenting features include chondritis of the nasal bridge, auricular chondritis, ocular inflammation and involvement of the bronchial tree. Its rarity often leads to considerable delay in establishing a diagnosis and poses a significant management challenge to clinicians, as no conventional guidelines exist. This review summarizes the clinical features of RPC and provides guidance for rheumatologists on making the diagnosis and assessing organ involvement. The current state of RPC management is reviewed, with a focus on the use of the anti-TNF-α agents in patients with pulmonary involvement, the leading cause of mortality and morbidity in RPC.

Relapsing polychondritis in systemic sclerosis: A rare vasculitic mimic

Article

Full-text available

Jan 2023

Introduction Relapsing polychondritis is a rare, immune-mediated disease characterised by inflammation of cartilaginous structures. Auricular chondritis, sparing the fatty lobule, is the most typical feature, followed by nose and laryngotracheal involvement. Albeit rare, neurologic involvement is reported with relapsing polychondritis. Cranial nerve involvement is the most frequent neurologic manifestation and is probably due to an underlying vasculitic process. Approximately one-third of relapsing polychondritis patients can overlap with other systemic diseases, including other autoimmune connective tissue diseases, but association with systemic sclerosis has very rarely been described. Case description A 63-year-old woman presented with acute new-onset severe dysphagia, accompanied by hoarseness and preceded by pain, swelling and erythema of the left pinna, unresponsive to antibiotics. She had a history of long-standing limited cutaneous systemic sclerosis. Cranial nerve examination revealed right-sided palatal palsy, and left vocal cord palsy was found on fibreoptic nasendoscopy. Magnetic resonance imaging of the head and neck showed bilateral enhancement of an extracranial segment of the glossopharyngeal and vagus nerves. Clinical features and imaging findings were consistent with relapsing polychondritis, which successfully responded to high-dose steroids. Conclusions This is a case of relapsing polychondritis mimicking progression of systemic sclerosis, showcasing its challenging features. It emphasises the importance of early diagnosis and prompt management with potential impact on the outcome, while highlighting the complex interplay between these two disease entities and vasculitic mechanisms, which may reflect the shared network of genetic predisposition across autoimmune rheumatic diseases.

Damage of cardiovascular system in patients with rare systemic diseases: example of recurrent polychondritis (case from practice)

Article

Dec 2022

A clinical case of recurrent polychondritis with damage to the cardiovascular system in the form of the development of acute coronary syndrome is presented. Recurrent polychondritis is a rare systemic presumably autoimmune disease that is affecting cartilage tissue. The main cause of high mortality in patients with systemic diseases is the early formation and accelerated progression of atherosclerotic vascular lesions that cause the development of myocardial infarction, stroke, chronic heart failure and sudden death. Recurrent polychondritis is characterized by clinical polymorphism. The diagnosis of the disease requires the interdisciplinary medical participation of at least five specialists.

Coexistence of Relapsing Polychondritis and Sickle Cell Disease in a Child

Article

Full-text available

Nov 2021

Relapsing polychondritis (RP) is a rare, severe connective tissue disease of unknown etiology affecting cartilaginous and proteoglycan-rich structures in an episodic and inflammatory manner. Approximately a third of RP cases occur in conjunction with another disease usually systemic autoimmune rheumatic disease, or myelodysplastic syndrome. Sickle cell disease (SCD) is a common inherited hematologic condition characterized by the inheritance of two abnormal hemoglobins, of which one is a hemoglobin S, presenting with severe acute and chronic complications from vaso-occlusive phenomena, which can be difficult to differentiate from RP. The pathogenesis of RP is poorly understood but suggests an autoimmune mechanism with a link to sickle cell disease yet to be established. Treatment is empiric with steroids, anti-inflammatory, and disease-modifying antirheumatic drugs being the mainstay of therapy. Severe complications occur despite treatment, with respiratory involvement being the most catastrophic. This case report reviews a complex case of RP in an 11-year-old girl with sickle cell disease (SF genotype) presenting with bilateral red painful eyes, a painful swollen left ear, and knee pain. Laboratory findings revealed elevated inflammatory markers with negative immune serology. A diagnosis of RP was made based on the patient's symptomatology, presentation, and fulfillment of 5 out of the 6 clinical features using McAdam’s criteria. Management was instituted with a myriad of conventional and biologic DMARDs and other anti-inflammatory medications with no significant improvement and the development of complications of airway obstruction from disease activity and osteoporotic fracture from steroid therapy and underlying hemoglobinopathy. In children, the diagnosis of RP is delayed or overlooked due to its low incidence, variability in clinical symptoms, or sharing similar clinical features with other coexisting disease entities. This article reports its occurrence in the pediatric population and highlights the difficulty in managing such cases as there are no defined standard treatment protocols. 1. Introduction Relapsing polychondritis (RP) is a rare, severe connective tissue disease with an episodic and progressive inflammatory nature involving cartilaginous structures, mainly the ear, nose, and laryngotracheobronchial tree. The disease also affects the joints, sclera, and various proteoglycan-rich tissues including the media of arteries [1]. Sickle cell disease (SCD) refers to a group of disorders of hemoglobin in which the sickle mutation is coinherited with a mutation at the other beta globin allele that reduces normal beta globin production. These include sickle cell anemia, sickle cell thalassemia, hemoglobin SC disease, and hemoglobin SF disease, which our patient had. SCD presents with severe acute and chronic complications involving many organs in the form of acute pain episodes, anemia, recurrent infections, and chronic end organ damage [2]. RP is common in patients aged 40–50 years but may occur at any age with a genetic link suggested by a significantly higher frequency of HLA-DR4 in patients with RP than in healthy individuals [1] and shows various immune responses directed against type 2 collagen and matrilin-1 [3]. Approximately 30% of RP cases coexist with other autoimmune inflammatory disorders with a small percentage being associated with nonrheumatic conditions [4]. However, the coexistence of RP and SCD has not been reported, and the management of such cases remains uncertain. RP is thought to be very rare among individuals of African descent with the majority of patients being of Caucasian origin [5]. Pediatric RP accounts for less than 5% of the cases reported, and it is characterized by frequent hospitalizations and emergency room visits, missed school attendance, and significant disability [6]. The most common clinical features of RP are chondritis and polyarthritis. Bilateral auricular chondritis is the most common feature observed in 90% of cases during the course of the disease, and this presents as painful, red, violaceous edema confined to the cartilaginous part of the ear. This leads to deformation of the ear following repeated flares and resembles the cauliflower ear of professional boxers. They can also present with either conductive or sensorineural hearing loss, saddle nose deformity, and laryngotracheobronchial involvement. Nonerosive asymmetric arthritis, ocular manifestations such as episcleritis, scleritis, uveitis, retinopathy, and conjunctivitis are also features of the disease [7]. Less frequently, patients can present with neurological involvement in the form of headaches, vasculitis, meningitis, and cerebral infarction, with IgA nephropathy, tubulointerstitial nephritis, and glomerulonephritis being some of the renal manifestations [7]. The diverse and nonspecific clinical features of RP alongside its relative rarity frequently lead to a diagnostic delay [8] which is associated with the lack of ear, nose, or joint involvement but presents with early signs of intermittent arthritis or eye involvement in such cases [4]. RP patients may develop significant disabilities during the course of the disease such as hearing and visual impairments, and 30–50% of patients suffer pulmonary complications [9, 10]. The diagnosis of RP is mainly clinical using a classification criteria developed by McAdam and later expanded on by Damiani-Levine and Michet, which includes recurrent inflammation of both auricular, nasal, laryngeal and tracheal cartilages, noninflammatory arthritis, inflammation of ocular structures, and cochlear or vestibular damage [4]. The diagnosis can also be made from a positive biopsy of the ear, nasal, or respiratory cartilage [4, 7]. There are no laboratory investigations specific for the diagnosis of RP and laboratory findings may be suggestive of ongoing inflammation or organ damage revealing the presence of anemia, thrombocytosis, leukocytosis, or a derangement in renal function or urinalysis signifying renal impairment [7]. In RP, serological tests such as the rheumatoid factor, antinuclear antibodies (ANA), complement levels, extractable nuclear antibodies (ENA), and antiphospholipid antibodies are usually negative, but may be positive in the presence of other associated autoimmune conditions [11]. ANA is usually negative and can occur in low titers. The presence of significant titers of ANA in a patient with RP connotes a possibility of an associated autoimmune disorder [7]. Antineutrophil cytoplasmic antibodies (ANCA) may be present in about 25% of patients with RP which can be an incidental finding or the presence of an ANCA-associated vasculitis or herald the beginning of one [3]. The management of RP is mainly empirical as there are no evidence-based guidelines for treatment. The aim of therapy is to reduce inflammation through immunosuppression and prevent multiorgan damage to cartilaginous structures [7]. Mild disease is treated with low dose steroids, NSAIDs, colchicine, and dapsone while high dose steroids and conventional DMARDs such as methotrexate, azathioprine cyclophosphamide, or mycophenolate mofetil are employed in severe life-threatening flares [4]. Biologic therapy agents such as infliximab, etanercept, and adalimumab appear to show efficacy in the treatment of RP that is resistant to conventional DMARDs [12]. The disease entity continues to baffle the scientific community because of its rarity, unknown etiopathogenesis, clinical diversity, unpredictable progression, and uncodified treatment [1]. Coexistence of SCD and RP, an autoimmune disorder, poses a diagnostic and therapeutic challenge as patients with these conditions can present with symptoms that are similar in both conditions, for instance, fever, polyarthritis, and multiorgan involvement. Therefore, early diagnosis of RP is important to define the best treatment modality which may include targeted biological therapy [13]. RP in the setting of sickle cell disease can be difficult to diagnose as a result of the nonspecific nature of symptoms and presentation; therefore, a high index of suspicion is needed to make the diagnosis early and institute treatment to prevent the significant morbidity and mortality associated with the disease, especially in the pediatric group. The purpose of this article is to highlight the fact that the disease can occur in the pediatric population and also in association with sickle cell disease. It also aims to reiterate the difficulty in managing such cases as there are no written protocols in the treatment of RP and that medications used in the treatment of RP can worsen SCD and its complications. We describe a case of relapsing polychondritis in an 11-year-old girl with sickle cell disease (SF genotype) who suffered severe respiratory tract involvement, which posed a considerable management challenge. This case is of significant interest due to the rarity of the condition, the young age of our patient, and the association with sickle cell disease; previously unreported in the literature compared to the known coexistence with autoimmune conditions such as vasculitis, systemic lupus erythematosus, and rheumatoid arthritis, and the hurdles encountered during treatment. 2. Case Report A 9-year-old young girl was referred to the rheumatology clinic from the ophthalmology unit as a case of systemic vasculitis with episcleritis. She presented with recurrent bilateral red painful eyes, a painful swollen left ear, and left knee pain in the preceding 10 months. A month prior to the presentation, she had developed an intermittent fever and reduced hearing. Three months prior to this, she had been admitted with painful swelling of the left knee and a fever, for which she was treated for septic arthritis of the left knee. Two years preceding the visit to the rheumatology clinic, she had also presented to the otorhinolaryngology unit on several occasions with complaints of hearing difficulty and was diagnosed with otitis externa. She has sickle cell disease-genotype SF and is a regular attendant at the pediatric sickle cell clinic. She did not suffer from an autoimmune condition, but had a first-degree cousin with systemic lupus erythematosus. She is the first of 2 children (her younger sibling is well) and has been unable to attend school due to arthralgia and difficulty hearing. 2.1. Clinical and Laboratory Findings On examination, there was mild pallor, axillary lymphadenopathy, and bilateral conjunctival injection with proptosis. The pinnae were swollen, erythematous, and tender, resembling “cauliflower ears” (Figure 1). The cardiorespiratory system examination was normal. There was acute synovitis of both knees with an old arthrotomy scar on the left (Figure 2).

Deformidade do nariz em sela em doenças reumatológicas: Uma revisão

Article

Full-text available

Dec 2023

Objetivo: Analisar a associação da deformidade do nariz em sela com doenças reumatológicas. Metodologia: Trata-se de uma revisão narrativa de literatura pautada na pergunta norteadora: “Qual a ocorrência de nariz em sela em pacientes com doenças reumatológicas?” A coleta de dados foi efetuada nas bases de dados BVS, PubMed e Scielo utilizando o operador booleano “OR” e os seguintes Descritores em Ciências da Saúde (DeCS): Doenças reumatológicas; Deformidades nasais adquiridas; Granulomatose com poliangiite; Policondrite recidivante e Vasculite associada a ANCA. A seleção das publicações obedeceu aos seguintes critérios de elegibilidade: artigos completos disponíveis eletronicamente, em português, inglês ou espanhol, publicados entre 2015 e 2022, cujos títulos e resumos mostraram-se congruentes com o propósito da revisão de literatura. Trabalhos destoantes do objetivo da pesquisa bem como teses, dissertações, monografias e resumos de anais não foram selecionados. Resultados: Os dados coletados foram submetidos a análise criteriosa resultando em uma amostra final de 22 artigos científicos. Os resultados obtidos corroboram as evidências da associação entre o nariz em sela e as doenças imunomediadas denominadas granulomatose com poliangiite e policondrite recidivante e permitiram o reconhecimento de novas correlações entre essa deformidade nasal e outras afecções reumatológicas, sendo elas granulomatose eosinofílica com poliangiite, sarcoidose e doença relacionada à IgG4. Conclusão: É necessária a produção de mais estudos científicos que avaliem a ocorrência de nariz em sela nas doenças reumáticas para ampliar as evidências e abrangência do assunto e elucidar os diagnósticos diferenciais associados ao nariz em sela.

Polychondritis Recidivans – A Case Report with a Review of the Literature

Article

Dec 2023

Relapsing Polychondritis Presenting With Hoarseness and Multisystem Involvement: A Diagnostic and Therapeutic Challenge

Article

Nov 2024

Relapsing Polychondritis with Tracheobronchial Involvement: A Detailed Description of Two Pediatric Cases and Review of the Literature

Article

Full-text available

Jan 2024

Relapsing polychondritis (RP) is a rare immune-mediated disease that primarily affects the cartilaginous structures of the ears, nose and airways. The clinical spectrum ranges from mild to severe disease characterized by progressive destruction of cartilage in the tracheobronchial tree leading to airway obstruction and acute respiratory failure. Early diagnosis is crucial to prevent irreversible airway damage and life-threatening complications. Due to its rarity and variability of symptoms, the diagnosis of RP is often delayed particularly in childhood. To address this and increase awareness of this rare disease, we present a detailed case report of two adolescent females affected by RP. We aim to describe the clinical findings, consequences of a delayed diagnosis and provide a review of the current literature.

Imaging features and clinical value of 18F-FDG PET/CT for predicting airway involvement in patients with relapsing polychondritis

Article

Full-text available

Oct 2023
ARTHRITIS RES THER

Background The clinical value of ¹⁸ F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) in assessing relapsing polychondritis (RP) with airway involvement remains controversial. This study aimed to investigate PET/CT features of RP with airway involvement and explore its clinical value in predicting disease pattern, severity and prognosis. Methods RP patients with airway involvement who underwent PET/CT from January 2010 to July 2022 were retrospectively reviewed. PET/CT features were analyzed both visually and semiquantitatively with the maximum standardized uptake value (SUVmax) and total lesion glycolysis (TLG). Patterns of airway involvement on PET were summarized. Correlations of SUVmax and TLG of the airway were made with spirometric indicators and serological inflammatory markers (CRP and ESR). In addition, long-term follow-up was conducted through questionnaires in regard to symptom control, subjective feeling, pulmonary function, and quality of life. Results Fifty-two cases were finally included. ¹⁸ F-FDG PET showed FDG-avid lesions with increased FDG uptake in the airway among 94.2% of the patients. Three patterns (focal, multifocal and diffuse patterns) were identified. TLG of the whole airway was lower in patients with previous therapy ( p = 0.046). Bronchoscopy was more sensitive in detecting tracheal abnormalities (90.7% vs.53.5%, p = 0.039) but less sensitive for peripheral airway lesions (65.1% vs. 79.1%, p = 0.046) compared with PET. SUVmax and TLG of the airway positively correlated with spirometry indicators (FEV1%pred, FEV1/FVC, MEF 50%pred, etc.) and serological inflammatory markers. Five patients died during the follow-up, with two deaths related to airway problems. Higher FDG uptake predicted worse subjective feeling, but not with symptom control or pulmonary function. Conclusion PET/CT is a valuable tool for RP with airway involvement, particularly in assessing peripheral airway lesions, and PET/CT related parameters are significantly associated with disease patterns, severity, and long-term outcomes.

Relapsing Polychondritis: A Rare Case Report

Article

Jun 2023

Relapsing polychondritis is an uncommon disorder of unknown cause characterized by inflammation of cartilage, predominantly affecting the ear, nose, and laryngotracheobronchial tree. The case under discussion is a 50-year-old female with a classical presentation of relapsing polychondritis with saddle nose deformity, bilateral auriculitis, and laryngotracheobronchomalacia with joint involvement.

Relapsing polychondritis presenting as otitis externa

Article

Full-text available

Jan 2023

Relapsing polychondritis remains a challenging diagnosis with cartilage inflammation being the hallmark disease pathology. Typical presentations include inflammation of auricular cartilage and joints, although multiple sites can be affected. Symptoms often overlap with other diseases and diagnosis is often delayed. Neurologic symptoms are rare and are attributed to CNS vasculitis. Here we report a rare case of relapsing polychondritis with neurological symptoms. This case illustrates both the challenges of diagnosis and the need to consider relapsing polychondritis in cases of cartilaginous inflammation.

Case Report: Lessons Learned From Subsequent Autologous and Allogeneic Hematopoietic Stem Cell Transplantations in a Pediatric Patient With Relapsing Polychondritis

Article

Full-text available

Mar 2022

Background Autologous hematopoietic stem cell transplantation (autoHSCT) is increasingly being recognized as a treatment option for severe refractory autoimmune diseases (AD). However, efficacy is hampered by high relapse rates. In contrast, allogeneic HSCT (alloHSCT) has high potential to cure AD, but is associated with significant morbidity and mortality, and data in AD are limited. Experience with autoHSCT in relapsing polychondritis, a rare episodic inflammatory disorder characterized by destruction of cartilage, is scarce and alloHSCT has not been described before. Case Presentation Here, we present a case of a 9-year-old girl who was diagnosed with relapsing polychondritis, with severe airway involvement requiring a tracheostomy. The disease proved to be steroid-dependent and refractory to a wide array of disease-modifying anti-rheumatic drugs and biologicals. After an autoHSCT procedure, the disease became inactive for a short period of time, until the patient experienced a relapse after 31 days, accompanied by repopulation of effector/memory CD8⁺ T cells. Because of persistent inflammation and serious steroid toxicity, including severe osteoporosis, growth restriction, and excessive weight gain, the patient was offered an alloHSCT. She experienced transient antibody-mediated immune events post-alloHSCT, which subsided after rituximab. She ultimately developed a balanced immune reconstitution and is currently still in long-term disease remission, 8 years after alloHSCT. Conclusion This case adds to the few existing reports on autoHSCT in relapsing polychondritis and gives new insights in its pathogenesis, with a possible role for CD8⁺ T cells. Moreover, it is the first report of successful alloHSCT as a treatment for children with this severe autoimmune disease.

Avaakchitiyam Adhyaya of Bhela Indriya Sthana - An explorative study

Article

Full-text available

Feb 2022

Avaakchitiyam is the twelfth chapter of Indriya Sthana of Bhela Samhita. The word ‘Avaakchitiyam’ refers to an inverted shadow (upside down) which is considered as a bad prognostic sign. Avaakchitiyam Adhyaya contains 17 verses dealing with various fatal conditions having poor prognosis. Previous research has established the prognostic potential of Indriya Sthana’s of Charaka Samhita and Bhela Samhita. Still further works are required as the concepts documented in ‘Avaakchitiyam Adhyaya’ of Bhela Indriya Sthana are poorly understood with no published literature. The present study aims to explore each and every verse of ‘Avaakchitiyam Adhyaya’ in terms of their prognostic importance with the help of contemporary medical literature. Various conditions such as orbital asymmetry with exophthalmos and enophthalmos, paralytic lagophthalmos, Bell’s palsy, ocular myopahties, eyebrow asymmetry associated with various neuro-ophthalmological disorders, peripheral neuropathies, neuropathic pain with mood disorders, scleroderma, saddle nose deformity, thermoregulatory disorders with autonomic neuropathies, myodesopsia, cenesthopathy, organic brain syndromes, cervical dystonia or torticollis with focal anhidrosis, septic shock, inflammatory bowel disease, visual snow syndrome, qualitative smell disorders, disease specific volatile organic compounds, specific anosmia, behavioural and psychological symptoms of dementia are documented in ‘Avaakchitiyam Adhyaya’ of Bhela Indriya Sthana. The present study provides insights for future research and action. Keywords: bhela samhita, behavioural and psychological symptoms of dementia, charaka samhita, indriya sthana, inflammatory bowel disease, visual snow syndrome

Early Recognition and Treatment of Relapsing Polychondritis

Article

Full-text available

Jan 2022

We describe the case of a 60-year-old Japanese man with relapsing polychondritis (RP). The patient was referred to Hamanomachi Hospital due to mild elevation of C-reactive protein and mild anemia on medical checkup without any symptoms. Body CT imaging showed thickened tracheal and bronchial walls with no active lesions in the lung. Precise physical examination revealed swelling in both ears. Bronchoscopy revealed redness and swelling of the tracheal and bronchial mucosa in the membranous lesion. Histologic examination of the bronchial biopsy showed inflammatory cell infiltration in the sub-mucosa with no vasculitis. Serum anti-type 2 collagen antibodies were found to be positive (33.9 EU/mL). Corticosteroid treatment improved his tracheochondritis. It is challenging to diagnose RP in the early stage due to its rarity and nonspecific symptoms. Airway involvement in RP is irreversible and the major cause of morbidity and mortality; hence, early recognition of airway involvement and treatment is warranted.

Extracorporeal high-frequency combined with contrast-enhanced ultrasound: a novel imaging method for detection and treatment evaluation of patients with cervical trachea-associated relapsing polychondritis

Article

Full-text available

Dec 2021

Background: This study aims to evaluate the clinical utility of extracorporeal high-frequency ultrasound (EHFU) combined with contrast-enhanced ultrasound (CEUS) for the diagnosis and treatment of cervical trachea-associated relapsing polychondritis (CT-RP). Methods: From January 2013 to January 2020, 24 patients with CT-RP diagnosed clinically and pathologically were retrospectively reviewed. We used EHFU to measure the thickness of trachea cartilage and the minimum internal transverse diameter of trachea and compared it with CT. The EHFU and CEUS imaging features were compared before and after treatment and used to evaluate the effects of therapy. Results: EHFU revealed the entire cervical tracheal cartilage (136 rings, of which 124 were abnormal). The average thickness of the 124 tracheal cartilage rings was 3.657±0.52 mm on EHFU and 3.32±0.76 mm on CT (t=1.482, P>0.05), while the diameter of the tracheal segment was 11.98±3.22 mm on EHFU and 10.8±2.92 mm on CT (t=1.005, P>0.05). After treatment, most patients (75%) showed no recurrence, and ultrasound revealed that the tracheal ring thickness was restored and the transverse diameter of the tracheal cavity was widened. Differences in EHFU measurements before and after treatment were statistically significant (both P<0.01). CEUS revealed that the tracheal cartilage layer was damaged in six cases (25%) and included structural destruction, deformation, and thinning. Follow-up evaluation revealed that the treatment outcomes of these cases were poor. The mean ultrasound examination time per patient was 10.0±2.3 min. Conclusions: EHFU combined with CEUS clearly and quickly revealed the CT-RP cervical tracheal wall, diameter of the trachea, and microstructural changes in tracheal cartilage. The effects of treatment can be reliably assessed by measuring reductions in tracheal cartilage thickening and echo changes before and after treatment. Dynamic monitoring of the condition provides timely and detailed information on cervical tracheal wall lesions and is valuable for clinical evaluation.

Meningoencephalitis in relapsing polychondritis: A case report

Article

Full-text available

Jun 2021
MEDICINE

Rationale: Aseptic meningoencephalitis is a rare central nervous system complication of relapsing polychondritis (RP). Patient: We report a 61-year-old Japanese male patient with spiking fever and impaired consciousness. Neurological examination revealed meningealirritation, and cerebrospinal fluid (CSF) examination showed lymphocytic pleocytosis with elevated protein (199 mg/dL) and interleukin-6 (3810 pg/mL). Serological analysis showed high levels of anti-type II collagen antibodies, and the result of auricular biopsy was consistent with the diagnosis of RP showing cartilage degeneration surrounded by inflammatory cell infiltrations. Diagnosis: A clinical diagnosis of RP was made according to the diagnostic criteria established by MacAdams et al. Intervention: Steroid pulse therapy (methylprednisolone 1000 mg, consecutive 3 days) followed by oral prednisolone (60 mg/day) resolved the patient's high fever and disturbance of consciousness. Outcomes: The patient rapidly improved after steroid treatments and has a normal quality of life under the maintenance dose of steroid plus methotrexate (4 mg/week). Lessons: RP-associated meningoencephalitis is a rare complication with significant morbidity and mortality. It should be considered and differentiated in patients with RP with unexplained spiking fever and impaired consciousness. In addition, the assessment of cerebrospinal fluid interleukin-6 levels may be useful to investigate the disease activity of RP-related meningoencephalitis. Further prospective studies are required to confirm this result.

Application of extracorporeal membrane oxygenation to treatment of serious tracheal stenosis caused by relapsing polychondritis: a case report and literature review

Article

Full-text available

Apr 2021
J INT MED RES

Relapsing polychondritis (RP) is a multisystemic rheumatic disease characterized by widespread and potentially destructive inflammatory lesions of the cartilage. The rarity of this disease and the lack of pathological diagnostic laboratory tests can occasionally lead to delayed diagnosis. We herein describe a 51-year-old woman with RP. She was sent to our hospital 4 days after the development of an upper respiratory tract infection with difficulty breathing. Her clinical condition significantly improved after the performance of extracorporeal membrane oxygenation support in an awake state, implantation of a tracheal stent, and administration of steroid therapy. Airway involvement of RP may be life-threatening. In this case, endotracheal intubation would have undoubtedly been very dangerous. Extracorporeal membrane oxygenation can be performed in an awake state to maintain oxygenation and improve the chance of survival.

Comparison of relapsing polychondritis patients with and without central nervous system involvement: A retrospective study of 181 patients

Article

Full-text available

Mar 2021
INT J IMMUNOPATH PH

The relapsing polychondritis (RP) patients with central nervous system (CNS) involvement were rare. We aimed to determine the clinical characteristics of RP patients with CNS involvement. The clinical data of 181 RP patients, hospitalized at Peking Union Medical College Hospital between December 2005 and February 2019, were collected. The patients were categorized into two subgroups: 25 RP patients with CNS involvement, and 156 RP patients without CNS involvement. The involvement of the ear was more frequent in RP patients with CNS involvement, compared with those of RP patients without CNS involvement ( P < 0.01). After controlling sex and the admission age, logistic regression analysis revealed hypertension (odds ratio = 4.308, P = 0.006) and involvement of eye (odds ratio = 5.158, P = 0.001) and heart (odds ratio = 3.216, P = 0.025) were correlated with RP patients with CNS involvement, respectively. In addition, pulmonary infection (odds ratio = 0.170, P = 0.020), tracheal involvement (odds ratio = 0.073, P < 0.01), and involvement of laryngeal (odds ratio = 0.034, P = 0.001), costochondral joint (odds ratio = 0.311, P = 0.013), sternoclavicular joint (odds ratio = 0.163, P = 0.017) and manubriosternal joint (odds ratio = 0.171, P = 0.021) were associated with RP patients without CNS involvement, respectively. In contrast to RP patients without CNS involvement, the incidence of ear involvement was higher in RP patients with CNS involvement. After controlling the potential confounding factor sex and the admission age, hypertension and involvement of eye and heart were related with RP patients with CNS involvement, respectively.

Relapsing Polycondritis

Chapter

Sep 2024

Relapsing polycondritis (RP) is a rare disease described for the first time a century ago under the term “polycondropathy” by Wartenhorst [1] and which owes its current name to Pearson et al. [2]. It is a systemic inflammatory immune-mediated disease that affects the cartilage tissues of ears, nose, and tracheobronchial tree but also the joints, eyes, inner ear, and cardiovascular system among several other systemic manifestations [3, 4]. It can present with different phenotypes. Some are life threatening such as the hematological phenotype associated with myelodysplasia and the respiratory one with predominant tracheobronchial involvement; other phenotypes are more benign, such as those characterized by isolated intermittent involvement of the cartilage of the nose and ears [5]. It is a very rare disease, with an estimated incidence of 0.7–3.7 per million person years [6–8]. A recent estimated prevalence of RP was in the United States 4.5 per million [9], while in the Hungarian population a prevalence of 20 per million has been calculated [8]. It appears to be ubiquitous across all ethnic groups although the majority of patients reported are of Caucasian origin and the disease is rare among sub-Saharan Africans [10]. It can appear at any age even although appears more frequently between the age of 40 and 50 years. For some authors there is a slight female predominance [11], while for others the disease affects both sexes similarly [10]. Predisposing genetic factors have been hypothesized and the occurrence of the disease in the same family has been reported [12].

Systematic Literature Review of the Presentation and Management of Pediatric Relapsing Polychondritis

Article

Aug 2024
Ann Otol Rhinol Laryngol

Introduction Pediatric Relapsing Polychondritis (RP) is a rare autoimmune disorder that causes inflammation and damage to cartilage in children. Common symptoms include pain, swelling and deformities in the ears, nose, trachea, joints, and eyes. The lack of research on the pediatric population necessitates further evaluation of the literature on pediatric RP to summarize existing patterns in presentation, management, and treatment. Methods A systematic review was conducted on PubMed and Embase from 1947 to April 2023 on RP in patients under 21 years old abiding by the 2020 PRISMA checklist. Only patient presentations meeting McAdam criteria for RP and including information on management were included. Results From the 304 initial studies, 54 studies were included for final analysis with a total of 68 patients, who were predominantly female (65%). With a median diagnostic delay of 1 year, the mean age of onset was 12 years old. The most common symptoms on presentation included bilateral auricular chondritis (69%), nasal cartilage inflammation (62%), and respiratory tract chondritis (63%). The most commonly reported information in the literature for the initial workup usually included CT/MRI (72%), bronchoscopy (57%), biopsy (51%), and labs (88%), which most commonly displayed elevated ESR (59%). The most common medications were corticosteroids (91%) and methotrexate (35%) and the most common procedural treatment was tracheostomy (38%). The most efficacious treatment options were monoclonal antibodies (87%, n = 15) and corticosteroids (66%, n = 62) used in 22% and 91% of patients, respectively. The most commonly used monoclonal antibody therapy was infliximab (13%, n = 9). Conclusion The most common presentation for pediatric RP includes chondritis of the ear, nose, and respiratory tract. The most effective treatment options include corticosteroids and monoclonal antibody therapy, such as infliximab. Our findings highlight increasing remission achieved with anti-rheumatic drugs and monoclonal antibody treatment, especially alongside corticosteroids.

Atteinte trachéo-bronchique de la polychondrite atrophiante et diagnostics différentiels

Article

May 2024
REV MAL RESPIR

JANUS KINASE INHIBITORS FOR RELAPSING POLYCHONDRITIS TREATMENT: A HYPOTHESIS

Article

Full-text available

Feb 2024

Neslihan Gokcen

Relapsing polychondritis (RP) is a rare autoimmune disease marked by recurrent episodes of inflammation impacting cartilaginous structures. The underlying mechanism has not been fully elucidated; however, comprehensive genetic and histopathological evaluations have revealed the involvement of specific genes, cell-mediated immunity, and humoral immunity in the pathogenesis of RP. The spectrum of symptoms associated with this condition ranges from mild manifestations to severe, life-threatening presentations. Treatment options vary depending on the disease severity. Non-steroidal anti-inflammatory drugs, colchicine, dapsone, and systemic corticosteroids are commonly utilized as first-line therapeutic options. Furthermore, cyclophosphamide, methotrexate, azathioprine, cyclosporine, and biological disease-modifying anti-rheumatic drugs are employed as second-line treatment. Nevertheless, there is insufficient data regarding the use of Janus kinase inhibitors (JAKi) in RP patients as a treatment option. This hypothesis suggests that JAKi may be a viable treatment option for relieving symptoms in these patients.

Policondrite recidivante e síndrome VEXAS: como esses caminhos se cruzam?

Article

Mar 2023

A policondrite recidivante é uma doença autoimune sistêmica rara caracterizada por crises recorrentes de inflamação de tecidos cartilaginosos e/ou ricos em proteoglicanos. Acomete ambos os sexos, com pico entre 45 e 55 anos de idade. As manifestações clínicas incluem sintomas constitucionais, condrite auricular e nasal, além de acometimento articular, dermatológico, ocular, cardiorrespiratório. A síndrome VEXAS (vacuoles, enzyme E1, X-linked, autoinflammatory, somatic) é, por sua vez, uma doença autoinflamatória adquirida, definida primeiramente em 2020. Acomete principalmente o sexo masculino, com pico após os 50 anos de idade. Apresenta várias manifestações clínicas semelhantes às descritas na policondrite recidivante. É nesse contexto que, no presente artigo, objetivamos descrever e revisar cada uma dessas doenças e, posteriormente, realizar uma comparação clinicolaboratorial entre elas. Unitermos: doença autoimune; doença autoinflamatória; policondrite recidivante; síndrome VEXAS.

Autoimmunity and Autoinflammation: Relapsing Polychondritis and VEXAS Syndrome Challenge

Article

Full-text available

Feb 2024
INT J MOL SCI

Relapsing polychondritis is a chronic autoimmune inflammatory condition characterized by recurrent episodes of inflammation at the level of cartilaginous structures and tissues rich in proteoglycans. The pathogenesis of the disease is complex and still incompletely elucidated. The data support the important role of a particular genetic predisposition, with HLA-DR4 being considered an allele that confers a major risk of disease occurrence. Environmental factors, mechanical, chemical or infectious, act as triggers in the development of clinical manifestations, causing the degradation of proteins and the release of cryptic cartilage antigens. Both humoral and cellular immunity play essential roles in the occurrence and perpetuation of autoimmunity and inflammation. Autoantibodies anti-type II, IX and XI collagens, anti-matrilin-1 and anti-COMPs (cartilage oligomeric matrix proteins) have been highlighted in increased titers, being correlated with disease activity and considered prognostic factors. Innate immunity cells, neutrophils, monocytes, macrophages, natural killer lymphocytes and eosinophils have been found in the perichondrium and cartilage, together with activated antigen-presenting cells, C3 deposits and immunoglobulins. Also, T cells play a decisive role in the pathogenesis of the disease, with relapsing polychondritis being considered a TH1-mediated condition. Thus, increased secretions of interferon γ, interleukin (IL)-12 and IL-2 have been highlighted. The “inflammatory storm” formed by a complex network of pro-inflammatory cytokines and chemokines actively modulates the recruitment and infiltration of various cells, with cartilage being a source of antigens. Along with RP, VEXAS syndrome, another systemic autoimmune disease with genetic determinism, has an etiopathogenesis that is still incompletely known, and it involves the activation of the innate immune system through different pathways and the appearance of the cytokine storm. The clinical manifestations of VEXAS syndrome include an inflammatory phenotype often similar to that of RP, which raises diagnostic problems. The management of RP and VEXAS syndrome includes common immunosuppressive therapies whose main goal is to control systemic inflammatory manifestations. The objective of this paper is to detail the main etiopathogenetic mechanisms of a rare disease, summarizing the latest data and presenting the distinct features of these mechanisms.

Paradoxical improvement of dyspnoea in relapsing polychondritis by glucocorticoid tapering

Article

Jan 2024

Successful Response to Golimumab in a Case of Relapsing Polychondritis Overlapping with Ulcerative Colitis

Article

Full-text available

Jan 2024

A 51-year-old Japanese man was diagnosed with left-sided ulcerative colitis (UC) at age 41. He was treated with mesalazine and azathioprine and maintained remission. At age 51, the patient developed bloody stools, abdominal pain, scleritis, arthritis, cough, bloody sputum, and pericardial effusion. Considering that pericardial effusion is an atypical extraintestinal complication of UC, and the patient met the diagnostic criteria for relapsing polychondritis (RP), a diagnosis of RP complicating a relapse of UC was made. Steroid therapy was administered, and both diseases improved. Golimumab, an anti-tumor necrosis factor-α inhibitor, was introduced as maintenance therapy for UC. All symptoms, including pericardial effusion, improved. Subsequently, no relapse of UC or RP was observed. As only a few cases of RP overlapping with UC have been reported and no treatment protocol has been established, we considered this case valuable and worthy of publication.

Predictors and prognosis of tracheostomy in relapsing polychondritis

Article

Nov 2023

Objective Airway obstruction can occur in patients with relapsing polychondritis with laryngeal involvement, occasionally requiring tracheostomy to avoid serious complications. Herein, we assessed the risk factors for tracheostomy and developed a risk prediction model. Methods Clinical characteristics of patients with relapsing polychondritis, with and without tracheostomy, were compared using multivariate logistic regression analysis to identify risk factors. A nomogram was developed to predict the population at risk of requiring tracheostomy. Results In total, 232 patients with relapsing polychondritis were reviewed, of whom 146 had laryngeal involvement. Among them, 21 underwent a tracheostomy. Multivariate logistic analysis identified ages ≤25 or ≥65 years [P < 0.001, odds ratio (OR) 24.584, 95% CI 5.310–113.815], laryngotracheal oedema (P < 0.001, OR 26.685, 95% CI 4.208–169.228) and pulmonary infection (P = 0.001, OR 18.834, 95% CI 3.172–111.936) as independent risk factors for tracheostomy. A nomogram with a C-index of 0.936 (95% CI 0.894–0.977) was established based on the multivariate analysis. Internal bootstrap resampling (1000 repetitions) confirmed sufficient discriminatory power with a C-index of 0.926. Decision curve analysis indicated a superior net benefit of the nomogram. Tracheostomy was associated with a significant increase in the in-hospital mortality rate (P = 0.021), but it did not affect the long-term survival rate (P = 0.706). Conclusion Tracheostomy is associated with an increase in the short-term mortality rate but does not affect the long-term survival rate. The nomogram developed in this study may help identify patients at high risk for tracheostomy and aid in clinical decision-making.

Bibliometric analysis of the global publication activity in the field of relapsing polychondritis during 1960–2023

Article

Aug 2023

Background Relapsing polychondritis (RP) is an inflammatory disease with significant individual heterogeneity that involves systemic cartilage tissues. This study aimed to perform a bibliometric analysis of RP-related publications to quantitatively assess the scholarly productivity in the field.Methods We extracted the RP-related original research articles and reviews published during 1960–2023 from the Web of Science database by using the keyword “relapsing polychondritis.” By using R, CiteSpace, VOSviewer, and SCImago Graphica, the bibliometric analysis was performed on the retrieved publications.ResultsA total of 1096 articles, consisting of 909 original research articles and 187 reviews, were identified. A mean annual growth rate of 6.71% was found in the number of RP-related publications during 1960–2022. The United States accounted for the highest number of publications (21.9%), exhibited the highest mean citation number per publication (40.7), and engaged in the most frequent academic collaboration. Three clusters of RP-related journals were identified: 1) otology, rhinology, and laryngology; 2) respiratory and radiology medicine; and 3) rheumatology. Journals with a focus on rheumatology issued the most publications, and most of the RP-related publications were from The Journal of Rheumatology (n = 27). Most of these publications were co-authored by Dr. Jean-Charles Piette (n = 19), who also had the highest H-index (13) among all the authors. The co-citation network analysis revealed 11 highly connected clusters of RP research and indicated the “VEXAS Syndrome” as a hotspot.Conclusion This overview of the RP research field comprehensively describes the progress in the field. The number of publications on RP has progressively increased but remains insufficient. The United States and European countries are at the forefront of RP-related research, and the journals related to rheumatology have covered the majority of publications. Additionally, several key topics for future investigations, such as "VEXAS Syndrome," have been identified. Key Points•We identified a mean annual growth rate of 6.71% in the number of the RP-related publications during 1960–2022.•The United States accounted for the majority of the publications, exhibited the highest mean citation number per publication, and engaged in the most frequent academic collaborations.•The journals of the publications were categorized into three clusters of research areas: 1) otology, rhinology, and laryngology; 2) respiratory and radiology medicine; and 3) rheumatology. Journals related to rheumatology issued the most publications, and most of the publications were from The Journal of Rheumatology•Most of the publications were co-authored by Dr. Jean-Charles Piette, who also had the highest scientific-research impact among the scholars in the field.•The co-citation network analysis revealed 11 highly connected clusters of RP research and indicated the “VEXAS Syndrome” as a key research area.

Immunvermittelte Erkrankungen

Chapter

Jul 2023

Frane Banovic

The Plot Thickens When the Trachea Is Thickened

Article

Jul 2023

TNFα inhibitor biosimilar associated with polychondritis. A case-based review

Article

Full-text available

Jun 2023
RHEUMATOL INT

Relapsing polychondritis (RP) is a rare autoimmune disease characterized by inflammation of the cartilage structures of the body with typical features of auricular chondritis, nasal and ocular inflammation, audio-vestibular damage, as well as respiratory tract manifestations. It is associated with several autoimmune diseases and many other disorders. Tumor necrosis factor alpha (TNFα) inhibitors treat many chronic inflammatory disorders. They have proven effective and relatively safe in many clinical trials and observational studies. However, several autoimmune phenomena and paradoxical inflammation have been described with TNFα inhibitors, among them RP. This report presents a 43-year-old man with psoriatic arthritis treated with ABP-501 (Amgevita), an adalimumab (ADA) biosimilar and who developed RP, 8 months after the initiation of the treatment. This, is the first report of RP development during TNFα inhibitors biosimilar. We concluded that rheumatologists dealing with patients treated with TNFα inhibitors (originators or biosimilars), should be aware of several paradoxical reactions which may emerge and RP, is one of them.

Connective Tissue Disease-Associated Interstitial Lung Disease

Chapter

Jun 2023

Various systemic autoimmune connective tissue disorders are associated with interstitial lung disease (ILD). Rheumatoid arthritis involves the joints; thoracic manifestations include ILD, diseases of the pleura, small or large airways, and pulmonary arteries. Systemic lupus erythematosus (SLE) is a relapsing–remitting chronic autoimmune disease that can affect multiple organs. Pulmonary involvement is more commonly seen in SLE than in any other connective tissue diseases. Sjogren syndrome occurs due to inflammatory lymphocytic infiltration of glandular and extraglandular organs. It is characterized by diminished function of exocrine glands that lead to eye and mouth dryness (sicca syndrome). ILD and small airway disease are the most common forms of lung involvement in Sjogren. Systemic sclerosis is a connective tissue disorder with multisystem involvement characterized by dysregulation of the immune system, and excess collagen formation leading to fibrosis of skin, vascular system, and multiple organs. ILD is more likely to develop in diffuse cutaneous systemic sclerosis than in the limited cutaneous entity. Dermatomyositis (DM) and polymyositis (PM) belong to a group of idiopathic inflammatory myopathies, which are connective tissue disorders that mostly involve the skeletal muscle. ILD is the major determinant of mortality and morbidity in patients with DM and PM. Ankylosing spondylitis (AS) is a chronic progressive autoimmune disorder involving the spine, the sacroiliac joints, and less commonly the peripheral joints. Pulmonary apical fibrosis is one of the commonest pulmonary features seen in AS. Relapsing polychondritis (RP) is a rare relapsing–remitting autoimmune disorder that causes inflammation predominantly of cartilaginous tissues. Airway involvement in the form of sub-glottic stenosis, tracheobronchomalacia, thickened and calcified tracheal wall with sparing of the posterior membranous wall, and tracheal stenosis which can be localized or diffuse are frequently seen pulmonary features in RP. Finally, mixed connective tissue disease comprises combination or overlap of systemic sclerosis, SLE, and DM/PM.KeywordsRheumatoid arthritisSystemic lupus erythematosusSjogren syndromeSclerodermaDermatomyositisAnkylosing spondylitisRelapsing polychondritisMixed connective tissue diseases

Relapsing polychondritis - A single Centre study in the United Kingdom

Article

May 2023
AUTOIMMUN REV

Introduction and objectives: Relapsing Polychondritis (RP) is a rare immune mediated inflammatory disorder that may result in damage and destruction of cartilaginous tissues. Patients and methods: We retrospectively analysed patients with a clinical diagnosis of RP. Patients were investigated using pulmonary function tests, dynamic high-resolution CT scans, bronchoscopy, laryngoscopy and/or PET-CT scans along with autoimmune serology. Patients had other specialist reviews when indicated. Results: We identified 68 patients with a diagnosis of RP, 55 (81%) were Caucasian, 8 (12%) Afro Caribbean, 4 (6%) Asian and 1 patient had Mixed Ethnicity. Twenty-nine (43%) had pulmonary involvement and in 16, pulmonary involvement was the initial presentation. The mean age at onset was 44 years (range 17-74). There was a mean diagnostic delay of 55 weeks. 66 (97%) patients received a combination of oral Prednisolone and disease modifying anti-rheumatic drugs. Twelve of 19 (63%) received biologics, with an initial good response, and 10 remain on treatment. 11 patients with respiratory collapse required CPAP to maintain airway patency. Twelve (18%) patients died due to RP and 9 had respiratory complications. Two patients developed myelodysplasia and one had lung carcinoma. In a multivariate regression analysis, the prognostic variables were ethnicity, nasal chondritis, laryngotracheal stricture and elevated serum creatinine. Conclusion: RP is a rare autoimmune condition often associated with significant delays in diagnosis and initiation of treatment. Pulmonary involvement in RP may cause significant morbidity and mortality due to organ damage. Disease modifying anti rheumatic drugs and biologics should be considered early in the disease course to minimise adverse effects of long-term corticosteroid therapy and organ damage.

Isolated Aortitis

Article

Apr 2023

The finding of aortitis, often incidentally noted on surgical resection, should prompt evaluation for secondary causes including large-vessel vasculitis. In a large proportion of cases, no other inflammatory cause is identified and the diagnosis of clinically isolated aortitis is made. It is unknown whether this entity represents a more localized form of large-vessel vasculitis. The need for immunosuppressive therapy in patients with clinically isolated aortitis remains unclear. Patients with clinically isolated aortitis warrant imaging of the entire aorta at baseline and regular intervals because a significant proportion of patients have or develop abnormalities in other vascular beds.

Convergence of Hematology and Rheumatology: A Case Study

Article

Full-text available

Mar 2023

Mariangel Prieto, MSN, APRN, FNP-BC, AOCNP

Advanced practitioners may frequently encounter patients who have a hematologic and rheumatologic diagnosis. These patients are usually managed by multiple specialists, including hematologists, rheumatologists, and dermatologists, given their broad symptomatology. Genetic testing may provide the answer to the constellation of symptoms and refractory symptoms that these patients exhibit.

Positron Emission Tomography Imaging in Vasculitis

Article

Feb 2023
CARDIOL CLIN

Systemic vasculitides comprise a group of autoimmune diseases affecting blood vessels. [18F]-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography (FDG-PET/CT) plays an important role in the diagnosis and therapeutic monitoring of vasculitides affecting large-sized and medium-sized vessels. FDG-PET/CT also provides complementary information to other vascular imaging tools. The resolution and sensitivity of newer generation scanners continues to increase, hereby improving the ability of FDG-PET/CT to accurately assess the full disease extent in patients with vasculitis. Novel tracers targeting specific immune cells will allow for more detailed detection of vascular infiltrates.

Recurrent polychondritis: a review of the literature and a clinical case description

Article

Full-text available

Feb 2023

Relapsing polychondritis (RPC) is a rare disease, its diagnosis presents certain difficulties. This is due to the absence of characteristic clinical manifestations at the initial stages of the disease, late diagnosis and difficulties in selecting adequate therapy.The article presents a review of the literature on the diagnosis and treatment of RPC, as well as a clinical case with tracheobronchial tree and other organ systems involvement in the absence of classical auricular involvement.

Respiratory Failure during BIS-Guided Sedation in a Patient with Relapsing Polychondritis: A Case Report

Article

Full-text available

Dec 2022

Relapsing polychondritis (RP) is a rare autoimmune disorder that causes inflammation and deterioration of cartilaginous structures such as the ears, nose, joints and laryngotracheobronchial tree. A 42-year-old man receiving treatment for RP underwent open reduction and internal fixation of a femur fracture under spinal anesthesia and with sedation by propofol and remifentanil. The level of sedation was monitored via a bispectral index (BIS), and maintained at between 60 and 80. At the end of the operation, he lost consciousness and displayed weak respiratory effort. During mask ventilation, the patient was judged to have respiratory failure due to high end-tidal CO2 (EtCO2) concentration and respiratory acidosis in an arterial-blood-gas analysis (ABGA). Ventilation through a properly inserted laryngeal-mask-airway or endotracheal intubation were impossible; instead, a surgical tracheotomy was performed. After recovering from respiratory failure with ventilatory support in the intensive care unit (ICU), he experienced the same symptoms three more times, requiring ventilatory support. He was discharged with bilevel positive-airway-pressure (BiPAP), after successful adaptation.

„Enthusiast meets the Experienced“: Pneumo meets … Rheumatologie

Article

Dec 2022

Many complex pulmonary diseases require close cooperation between pneumologists and rheumatologists. For example, almost every rheumatic systemic disease can be accompanied by connective tissue disease-associated interstitial lung disease (CTD-ILD). This often significantly negatively affects the prognosis. Pneumologists and rheumatologists are jointly called upon in the diagnostic process, especially in the delineation of drug toxicities and in therapeutic decisions. The same applies to various indirect forms of involvement of the lungs and pleura in patients with rheumatic diseases. Nodules and pleural effusions frequently occur but the diagnostic and therapeutic classification can be challenging. Pulmonary artery embolisms can also occur more frequently in certain diseases, such as in antiphospholipid antibody syndrome (APS) and Behçet’s disease. Sometimes severe bronchopathy develops in rheumatoid arthritis, recurrent polychondritis (RP) and granulomatosis with polyangiitis (GPA). Pulmonary hypertension can also occur in various rheumatic diseases and the classification into one of the five groups is therapeutically important.

Relapsing Polychondritis in a Patient With Auricular Chondritis and Inflammatory Bowel Disease: A Case Report With Literature Review

Article

Full-text available

Nov 2022

Relapsing polychondritis (RP) most commonly presents as inflammation and degeneration of cartilaginous tissue in the auricles, nasal septum, and lungs (in severe instances). RP is a rare autoimmune condition associated with other autoimmune diseases in 30% of cases. The prevalence of gastrointestinal involvement with RP is tenuous; however, there is a growing collection of case studies associating auricular chondritis with concomitant inflammatory bowel disease (IBD), including both ulcerative colitis and Crohn's disease. We report the case of a 35-year-old patient presenting with autoimmune pancreatitis, with a past medical history of Crohn's disease, primary sclerosing cholangitis (PSC), and suspected RP. Although RP is rare, the disease's multiple clinical presentations and recurrent episodic nature can cause significant diagnostic delays and are often overlooked by physicians. Thus, low disease prevalence may be due to under-recognition and under-reporting of disease symptoms. As RP is a clinical diagnosis, increased awareness of the disease presentation and clinical characteristics may increase disease recognition and improve treatment outcomes.

Clinical Characteristics of Our Follow-Up Patients with the Rare Disease Relapsing Polychondritis: A Single-Center Study

Article

Jul 2022

Relapsing polychondritis (RP) is an immune-mediated, systemic inflammatory and degenerative disease that affects cartilaginous structures particularly the ears, nose, eyes, joints, and respiratory tract and other tissues in the body. RP targets non-cartilaginous structures such as skin, kidney, heart, and the central nervous system in addition to cartilage-containing structures. Since it is a rare disease, data on its epidemiology are insufficient. It is prevalent between the ages of 40 and 60. Men and women are affected equally. Clinical characteristics vary among patients. The disease's rarity and broad clinical spectrum frequently result in misdiagnosis or delayed diagnosis. To prevent related complications and death, and to improve prognosis, early diagnosis and timely treatment of RP are crucial. Glucocorticoids, dapsone, disease-modifying antirheumatic drugs (DMARDs), and biologics are available as treatment options. The prognosis, such as the clinical presentation, varies depending on the extent of organ damage. 10 patients with RP had a mean onset age of 49.5±4.1. The median time between symptom onset and diagnosis was 3 months (2-60). 80% of the patients were male. The most frequent clinical manifestation is auricular chondritis (100%). During treatment, oral prednisolone was administered to all patients at least once. Intravenous methylprednisolone was administered to two patients. Due to ineffectiveness of DMARDs, one patient was switched to infliximab. A patient died due to pneumosepsis. This article aims to increase clinicians' awareness of this rare disease that can affect multiple systems by providing an overview of its pathogenesis, clinical course, diagnosis, and treatment.

Associations between autoimmune dysfunction and pollutants in Akwesasne Mohawk women: Dichlorodiphenyltrichloroethane and polychlorinated biphenyl exposure

Article

Jun 2022

Background: Pollutant exposures, including polychlorinated biphenyls (PCBs) and dichlorodiphenyltrichloroethane (DDT), have been found to disrupt normal immune function. Native American communities are disproportionately affected by autoimmune dysfunction and are more likely to be exposed to harmful pollutants than the general population. Objective: To determine the association between autoimmune dysfunction and pollutant exposure levels, this study evaluates the statistical relationship between the presence of autoimmune dysfunction and pollutant exposure. Methods: Information was collected from Akwesasne Mohawk women (n = 182), 21-39 years of age, between 2009 and 2013. Data collection included anthropometric measurements, medical diagnoses of autoimmune disease and symptoms of autoimmune dysfunction in the medical record, and blood draws for measurement of pollutants. Multivariate analyses determined the association between toxicant exposure and autoimmune dysfunction. Results: Toxicant p,p'-DDE was positively associated with an almost two-fold risk of autoimmune dysfunction. p,p'-DDE and PCB congeners 32, 136, and 138 were positively associated in a multivariate analysis with an autoimmune diagnosis. Conclusions: Pollutant exposures, specifically to p,p'-DDE and some PCB congeners, are common exposures that are associated with autoimmune dysfunction and autoimmune disease, although there are other factors and causes related to autoimmune dysfunction incidence.

Risk factors for the recurrence of relapsing polychondritis

Article

Full-text available

May 2022
ARTHRITIS RES THER

Background Although the survival rates of patients with relapsing polychondritis (RP) have increased remarkably, the high recurrence rate remains a significant concern for physicians and patients. This retrospective study aimed to investigate the risk factors for RP recurrence. Methods Patients with RP who presented to Kyoto University Hospital from January 2000 to March 2020 and fulfilled Damiani’s classification criteria were included. Patients were classified into recurrence and non-recurrence groups. Risk factors for RP recurrence were analysed using a Cox proportional hazards model, and Kaplan–Meier survival curves were drawn. Results Thirty-four patients were included. Twenty-five patients (74%) experienced 64 recurrences (mean: 2.56 recurrences per patient). The median duration before the first recurrence was 202 [55−382] days. The median prednisolone dose at the initial recurrence was 10 [5−12.75] mg/day. Tracheal involvement was significantly more frequent in the recurrence group at the initial presentation (44.0% vs. 0.0%, p =0.0172) than in the non-recurrence group, and pre-treatment C-reactive protein levels were significantly higher in the recurrence group than in the non-recurrence group (4.7 vs 1.15 mg/dL, p =0.0024). The Cox proportional hazards model analysis revealed that tracheal involvement (hazard ratio [HR] 4.266 [1.535−13.838], p =0.0048), pre-treatment C-reactive protein level (HR 1.166 [1.040−1.308], p =0.0085), and initial prednisolone monotherapy (HR 4.443 [1.515−16.267], p =0.0056) may be associated with recurrence. The median time before the initial recurrence was significantly longer in patients who received combination therapy with prednisolone and immunosuppressants or biologics (400 vs. 70 days, p =0.0015). Conclusions Tracheal involvement, pre-treatment C-reactive protein level, and initial prednisolone monotherapy were risk factors for recurrence in patients with RP. Initial combination therapy with prednisolone and immunosuppressants may delay recurrence.

Recurrent Eye and Ear Pain in an Older Patient

Article

May 2022

Continuous Use of Etanercept During Pregnancy Does Not Affect TNF-Alpha Levels in Umbilical Cord Blood

Article

Full-text available

Mar 2022
Biologics

Masayuki Nishide,* Mayu Yagita,* Atsushi Kumanogoh Department of Respiratory Medicine and Clinical Immunology, Graduate School of Medicine, Osaka University, Osaka, Japan*These authors contributed equally to this workCorrespondence: Masayuki Nishide, Department of Respiratory Medicine and Clinical Immunology, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita, Osaka, 565-0871, Japan, Tel +81-6-6879-3831, Fax +81-6-6879-3839, Email nishide@imed3.med.osaka-u.ac.jpAbstract: TNF-alpha-targeted therapies during pregnancy is a topic of interest in rheumatology. Etanercept (ETN) is expected to have lower transplacental transfer, however, clinical evidence is lacking on the usefulness and safeness of continuing etanercept throughout pregnancy. We here described the first reported case of relapsing polychondritis where continuous use of ETN throughout pregnancy was required. The patient was a pregnant Japanese woman who presented with bilateral ear cartilage redness, swelling, saddle nose and severe subglottic oedema. Due to severe systemic and life-threatened disease, we decided to continue using ETN throughout pregnancy and resulted in successful vaginal delivery. The treatment with ETN was successful and TNF-alpha levels in umbilical cord blood were not affected. The infant did not have any signs of chondritis although levels of anti-type 2 collagen antibodies in maternal and umbilical cord blood were similar, suggesting that anti-type 2 collagen antibodies crossed the placenta. This case is an important clinical experience that strengthens the safety to continue ETN during the entire pregnancy if necessary.Keywords: etanercept, relapsing polychondritis, pregnancy, TNF-alpha, anti-type 2 collagen antibody

Ultrasonography of auricular cartilage is a potential tool for diagnosing relapsing polychondritis and monitoring disease activity

Article

Full-text available

Dec 2021

Aim: To assess the clinical utility of ultrasonography in the diagnosis and monitoring of disease activity in relapsing polychondritis (RP). Methods: Auricular and nasal chondritis of 6 patients with RP were assessed by ultrasonography before treatment initiation. Changes in the ultrasonographic and clinical findings and serum inflammatory markers were longitudinally assessed. Ultrasonography was also performed in 6 patients with repeat ear trauma, 6 patients with auricular cellulitis and 6 healthy controls for comparison among groups. Results: In all cases of RP, ultrasonographic findings before treatment revealed low-echoic swollen auricular and nasal cartilage and perichondral soft-tissue with increased power Doppler signals (PDS) corresponding to biopsy findings. After 2-month treatment with prednisolone (PSL) combined with methotrexate, clinical and serum inflammatory markers were completely resolved. Although swollen perichondral soft-tissue, cartilage and PDS on auricular ultrasonography were also significantly improved, PDS remained in 2 of 6 cases, which showed flare early after tapering PSL. Finally, ultrasonographic findings of RP were substantially differentiated between patients with repeat trauma and cellulitis and healthy controls based on the thickness of soft tissue around the cartilage, PDS and subperichondral serous effusion. Conclusion: Assessment of RP lesions by ultrasonography is useful for the evaluation of cartilaginous lesions and monitoring of disease activity, especially when considering the treatment response and the timing of drug tapering.

Relapsing polychondritis: state-of-the-art review with three case presentations

Article

Sep 2021

Background: relapsing polychondritis (rpc) is a complex immune-mediated systemic disease affecting cartilaginous tissue and proteoglycan-rich organs. The most common and earliest clinical features are intermittent inflammation involving the auricular and nasal regions, although all cartilage types can be potentially affected. The life-threatening effects of rpc involve the tracheobronchial tree and cardiac connective components. Rpc is difficult to identify amongst other autoimmune comorbidities; diagnosis is usually delayed and based on nonspecific clinical symptoms with limited laboratory aid and investigations. Medications can vary, from steroids, immunosuppressants, and biologics, including anti-tnf alpha antagonist drugs. Method: information on updated etiology, clinical symptoms, diagnosis, and treatment of rpc has been obtained via extensive research of electronic literature published between 1976 and 2019 using pubmed and medline databases. English was the language of use. Search inputs included ‘relapsing polychondritis,’ ‘polychondritis,’ ‘relapsing polychondritis symptoms,’ and ‘treatment of relapsing polychondritis.’ published articles in english that outlined and reported rpc’s clinical manifestations and treatment ultimately met the inclusion criteria. Articles that failed to report the above and reported on other cartilaginous diseases met the exclusion criteria. Result: utilizing an extensive overview of work undertaken in critical areas of rpc research, this review intends to further explore and educate the approach to this disease in all dimensions from pathophysiology, diagnosis, and management. Conclusion: RPC is a rare multi-systemic autoimmune disease and possibly fatal. The management remains empiric and is identified based on the severity of the disease per case. The optimal way to advance is to continue sharing data on RPC from reference centers; furthermore, clinical trials in randomized control groups must provide evidence-based treatment and management. Acquiring such information will refine the current knowledge on RPC, which will improve not only treatment but also diagnostic methods, including imaging and biological markers.

FDG-PET/CT and Auricular Cartilage Biopsy Are Useful for Diagnosing with Relapsing Polychondritis in Patients without Auricular Symptoms

Article

Full-text available

Sep 2021

Relapsing polychondritis (RP) is a rare autoimmune inflammatory disease characterized by recurrent inflammation and destruction of cartilage. Although auricular chondritis is a characteristic finding in RP, it can be difficult to diagnose in the absence of auricular symptoms. A 64-year-old Japanese male was referred to our hospital with fever and respiratory distress. Contrast-enhanced computed tomography (CT) revealed bronchial wall thickening and we suspected RP; however, he had no auricular symptoms and did not meet the diagnostic McAdam criteria for RP, so we used 18F-fluorodeoxyglucose positron emission tomography/CT (FDG-PET/CT) to search for other cartilage lesions. This analysis revealed FDG accumulation not only in the bronchial walls, but also in the left auricle. Instead of a bronchial biopsy using a bronchoscope, we performed a biopsy of the left auricular cartilage, which is considered a relatively less invasive site. Even though the auricle was asymptomatic, the pathology results revealed chondritis. He was diagnosed with RP, and his symptoms rapidly improved with corticosteroid therapy. A biopsy of asymptomatic auricular cartilage may be useful in the diagnosis of RP. FDG-PET/CT is a powerful tool for the early diagnosis of RP, identifying inflammatory areas even in the absence of symptoms, and guiding the selection of appropriate biopsy sites.

Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis

Article

Full-text available

Aug 2021

Background: Relapsing polychondritis (RP) is an uncommon autoimmune condition that impacts cartilaginous structures involving the ears, nose, respiratory tract, and joints. Its etiology is unknown; however, it may be associated with other systemic autoimmune diseases, malignancy, and rarely with human immunodeficiency virus (HIV) infection. RP has a variable pattern at presentation and may be associated with constitutional symptoms such as fever and arthralgia, in addition to various auricular, ocular, respiratory, and cardiovascular manifestations. Auricular and ocular signs are the most common presenting features; however, idiopathic orbital inflammatory syndrome is considered a rare manifestation of the disease. Systemic corticosteroids are the mainstay of treatment, but immunomodulatory therapy may be required for refractory cases. Case report: We present a challenging case of RP in a 58-year-old female with HIV controlled on highly active anti-retroviral therapy (HAART) and stable chronic reactive arthritis on sulfasalazine who developed unilateral auricular chondritis associated with contralateral idiopathic orbital inflammation and scleritis as well as worsening arthralgia. She was initially treated empirically with antibiotics, without clinical improvement. Infectious workup was unrevealing, and other diagnostic possibilities were meticulously excluded. Clinical suspicion for RP ultimately led to appropriate therapy with corticosteroids and subsequent immunosuppression with methotrexate, resulting in clinical improvement and allowing for gradual tapering of steroids. Conclusion: RP is an uncommon multisystem disorder that may occur in the setting of other underlying chronic illnesses, as seen in our patient. It has a variable presentation and course with no diagnostic laboratory tests, and thus clinical suspicion is imperative for appropriate diagnosis and management.

Clinical patterns and the evolution of relapsing polychondritis based on organ involvement: a Chinese retrospective cohort study

Article

Full-text available

Dec 2021
ORPHANET J RARE DIS

Background Relapsing polychondritis (RPC) is a rare autoimmune disease and its early diagnosis remains challenging. Defining the clinical patterns and disease course may help early recognition of RPC. Results Sixty-six males and 60 females were included in this study. The average age at onset were 47.1 ± 13.8 years and the median follow-up period was 18 months. Correlation analysis revealed a strong negative correlation between airway involvement and auricular chondritis (r = − 0.75, P < 0.001). Four distinct clinical patterns were identified: Ear pattern (50.8%), Airway pattern (38.9%), Overlap pattern (4.8%) and Airway-Ear negative pattern (5.6%), and patients with Ear pattern and Airway pattern were further divided into limited and systemic form of RPC (27.8% with limited form of Ear pattern and 24.6% with limited form of Airway pattern initially). During follow-up, a minority of patients with Ear pattern and Airway pattern progressed into Overlap pattern, and some Airway-Ear negative pattern patients progressed into Ear pattern. While a large majority of limited RPC patients remained limited form during follow-up, a minority of limited RPC patients progressed into systemic form. Patients with Ear pattern had the highest survival rate and relatively lower inflammatory status. Conclusions RPC patients can be categorized as 4 different clinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement. The clinical patterns and presenting forms may evolve during follow-up. Our findings may facilitate early recognition of this rare disease.

Sudden Sensorineural Hearing and Vestibular Loss in a Case of Relapsing Polychondritis

Article

Apr 2021

Objectives To report a case of profound bilateral sensorineural hearing and vestibular loss from relapsing polychondritis and hearing outcomes after cochlear implantation. Methods Case report and literature review. Results A 43 year-old woman developed sudden loss of hearing and balance that progressed over several weeks to bilateral, profound hearing and vestibular loss. Steroid treatments were ineffective. She underwent vestibular physical therapy and left cochlear implantation. About 10 months after her initial presentation, she developed erythema, warmth, swelling, and pain of the left auricle sparing the lobule, flattening of the bridge of her nose, and right ankle swelling, warmth, and skin erythema. A biopsy of the left auricle revealed histopathologic findings consistent with relapsing polychondritis. She was treated with high dose prednisolone. The ear inflammation resolved, however, despite excellent auditory response to pure tone thresholds, the patient reported no improvement in speech perception after cochlear implantation. Conclusions Relapsing polychondritis can present with rapidly progressive, profound loss of hearing and vestibular function. Hearing outcomes after cochlear implantation can include poor speech discrimination despite good pure tone detection thresholds.

Relapsing Polychondritis with Central Nervous System Involvement: Experience of Three Different Cases in a Single Center

Article

Full-text available

Nov 2016

Chan Hong Jeon

Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death. The second patient had acute cognitive dysfunction due to limbic encephalitis. He was treated with steroid pulse therapy and recovered without sequelae. The third patient suffered aseptic meningitis that presented as dementia, which was refractory to steroid and immune suppressive agents. We also reviewed literature on CNS complications of RP.

Incidence and mortality of relapsing polychondritis in the UK: a population-based cohort study

Article

Full-text available

Jul 2015

Relapsing polychondritis is a rare disease characterized by cartilage inflammation. Our aim was to estimate the incidence, prevalence and mortality of relapsing polychondritis and describe the clinical features of relapsing polychondritis in a large population. All participants diagnosed with relapsing polychondritis were sampled from the Clinical Practice Research Datalink. Prevalence and incidence rates for 1990-2012 were estimated. Relative mortality rates were estimated in a time-to-event framework using reference UK life tables. A questionnaire validation study assessed diagnostic accuracy. There were 117 participants with relapsing polychondritis ever recorded. Fifty (82%) of 61 cases were validated by a physician and unconfirmed cases were excluded. The analysis included 106 participants (42 men, 64 women) diagnosed with relapsing polychondritis. The mean age (range) at diagnosis in men was 55 (range 17-81) years and in women 51 (range 11-79) years. The median interval from first symptom to diagnosis was 1.9 years. The incidence of relapsing polychondritis between 1990 and 2012 was 0.71 (95% CI 0.55, 0.91) per million population per year. There were 19 deaths from any cause. There were 16 observed deaths eligible for survival analysis and 7.4 deaths expected for the UK population of the same age, sex and period. The standardized mortality ratio was 2.16 (95% CI 1.24, 3.51), P < 0.01. Respiratory disease, cardiac conditions and cancer were the most frequent causes of death. The incidence of relapsing polychondritis may be lower than previously estimated, and diagnostic misclassification and delay are common. Mortality in relapsing polychondritis is more than twice that of the general population. © The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Limbic encephalitis associated with relapsing polychondritis responded to infliximab and maintained its condition without recurrence after discontinuation: A case report and review of the literature

Article

Full-text available

Aug 2014
Nagoya J Med Sci

Central nervous system (CNS) manifestations are rare complications of relapsing polychondritis (RP). The majority of patients respond well to glucocorticoid therapy, but need to maintain it. Some patients are refractory to initial glucocorticoid therapy and to additional immunosuppressants, and end up with an outcome worse than at therapy initiation. The standardized therapeutic protocol for this condition has not been established. The effects of anti-tumor necrosis factor (TNF) -α agents have been reported recently. We experienced a patient with RP and limbic encephalitis who was refractory to initial high-dose glucocorticoid, but subsequently responded to infliximab and did not show deterioration of signs and symptoms after stopping therapy. We report this case together with a systematic literature review. This is the first case report of RP with CNS manifestations successfully treated by an anti-TNF-α agent without recurrence after discontinuation.

Relapsing polychondritis: A review

Article

Full-text available

Jul 2013
CLIN RHEUMATOL

Relapsing polychondritis is a rare multisystem disease involving the cartilaginous and proteoglycan rich structures. The spectrum of clinical presentations may vary from intermittent episodes of painful and often disfiguring auricular and nasal chondritis, to occasional organ or even life-threatening manifestations like airway collapse. There is lack of awareness about this disease due to its rarity. Relapsing polychondritis disease activity index has recently been validated and may help in clinical decision making and research. This article reviews the literature on this disease entity.

Relapsing polychondritis: An uncommon cause of dementia

Article

Full-text available

Feb 2009

Relapsing polychondritis Is a rare disorder that is characterised by recurrent and progressive inflammation of cartilaginous structures. Nervous system involvement in relapsing polychondritis has been described. We describe two cases of relapsing polychondritis with subacute dementia.

Blue ear sign in relapsing polychondritis

Article

Full-text available

Feb 2011

Sustained response to tocilizumab, anti-interleukin-6 receptor antibody, in two patients with refractory relapsing polychondritis

Article

Full-text available

Mar 2009

Autologous stem-cell transplantation in refractory autoimmune diseases after in vivo immunoablation and ex vivo depletion of mononuclear cells

Article

Full-text available

Feb 2000
Arthritis Res

Statement of findings Autoimmune diseases that are resistant to conventional treatment cause severe morbidity and even mortality. In the present study we demonstrate that complete remissions can be achieved in refractory polychondritis and systemic lupus erythematosus (SLE), even at advanced stage, with the use of autologous stem-cell transplantation (SCT). Remissions persisted after reconstitution of the immune system. In the treatment of advanced systemic sclerosis (SSc), stable disease may be achieved with autologous SCT.

Relapsing Polychondritis: A Review and Guide for the Dermatologist

Article

Oct 2016

Relapsing polychondritis, or RP, is a rare connective tissue disease characterized by relapsing-remitting destructive inflammation of the cartilaginous and other proteoglycan-rich structures in the body. Given the relatively low incidence of RP, a concise clinically relevant guide, focusing on the cutaneous manifestations of this serious disease, is lacking. In this review, we provide the dermatologist with an approach to diagnosing RP and a guide to its initial work-up, and management. We close with an overview of the currently available treatment modalities for RP.

Sustained response to infliximab in 2 patients with refractory relapsing polychondritis

Article

Jun 2003

Erratum: Dementia in a patient with non-paraneoplastic limbic encephalitis associated with relapsing polychondritis (Pathology (2006) 38, (596-599))

Article

Apr 2007

Relapsing polychondritis. Case report

Article

May 1973
PLAST RECONSTR SURG

F R Jones

A case of relapsing polychondritis is reported, in which the nasal deformity was improved with a silicone implant.

RELAPSING POLYCHONDRITIS

Article

Mar 1973
PLAST RECONSTR SURG

Frederick R. Jones

Relapsing Polychondritis

Article

Jul 1998

David E. Trentham

Relapsing polychondritis

Article

Mar 2014

Relapsing polychondritis (RP) is a rare disease in which recurrent bouts of inflammation, in some cases followed by destruction, affect the cartilage of the ears, nose, larynx, and tracheobronchial tree. At presentation, however, arthritis is the most common manifestation and more than half the patients have no evidence of chondritis. The subsequent development of chondritis provides the correct diagnosis in patients who present with polyarthritis, ocular inflammation, or skin or audiovestibular manifestations of unknown origin. A concomitant autoimmune disease is present in one-third of patients with RP. The pathogenesis of RP involves an autoimmune response to as yet unidentified cartilage antigens followed by cartilage matrix destruction by proteolytic enzymes. The diagnosis rests on clinical grounds and can benefit from use of Michet's criteria. Anti-collagen type II and anti-matrilin-1 antibodies are neither sensitive nor specific and consequently cannot be used for diagnostic purposes. In addition to the physical evaluation and laboratory tests, useful investigations include dynamic expiratory computed tomography, magnetic resonance imaging, Doppler echocardiography, and lung function tests. Bronchoscopy has been suggested as a helpful investigation but can worsen the respiratory dysfunction. The treatment of RP is not standardized. The drug regimen should be tailored to each individual patient based on disease activity and severity. Glucocorticoid therapy is the cornerstone of the treatment of RP and is used chronically in most patients. Immunosuppressive agents are given to patients with severe respiratory or vascular involvement and to those with steroid-resistant or steroid-dependent disease. Methotrexate is often effective. Cyclophosphamide is used in severe forms.

Clinical analysis of 15 patients with relapsing auricular polychondritis

Article

Mar 2013

Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology. The disease is characterized by episodic inflammation and destruction of cartilaginous and connective tissue structures, including the ear, eye, nose, larynx, trachea, bronchi, joints, skin, heart valves, and aorta. As the symptoms of RP are diverse and complex, it is easily misdiagnosed. The aim of this paper was to improve the understanding of the clinical features of RP, thereby facilitating its early diagnosis. Fifteen patients with RP were analyzed retrospectively and the relevant literature reviewed. The number of patients presenting with auricular chondritis was 13, while two presented with polyarthritis. Among them, the treatment of 2 RP patients with respiratory tract involvement failed and 1 patient died. Eleven patients with RP (73 %) were initially misdiagnosed. RP involves cartilage and connective tissue. The prognosis for patients with respiratory tract involvement is poor. RP causes episodic and progressive inflammation of cartilage throughout the body and is associated with a variety of clinical manifestations. Early diagnosis of RP depends on a thorough understanding of its clinical features.

Biologics in relapsing polychondritis: A case series

Article

Sep 2013

To describe the effects of biologics in an unbiased series of relapsing polychondritis cases. We extracted all the cases encoded 'polychondritis' from the computerized medical files of our department. The relapsing polychondritis diagnosis was confirmed using Damiani's criteria. Patients treated with biologics were evaluated for efficacy and adverse drugs reactions until October 2012. Nine patients were exposed to 22 biologics as corticosteroid-sparing drugs. Biologics were used at the same doses as in rheumatoid arthritis. Mean duration of exposure to biologics was 28 months. A TNF-antagonist was most frequently used as first-line biologic therapy (7/9), leading to partial or complete efficacy in six cases (85.7%). Loss of efficacy occurred in 5 cases. Abatacept (n=3) and tocilizumab (n=2) were effective as second-line biologic therapy while anakinra (n=2) and certolizumab (n=1) were not. Seven serious adverse drug reactions occurred, including 5 infections. TNF-α antagonists may be proposed earlier in relapsing polychondritis to spare corticosteroids. Switching to another biologic can be proposed in case of loss of efficacy. Tocilizumab or abatacept can be proposed as third-line therapy. The benefit-to-risk ratio of biologics in relapsing polychondritis should be evaluated prospectively.

A case of saddle nose deformity caused by relapsing polychondritis: A long-term follow-up report after iliac bone grafting

Article

Jun 2013

Successful Treatment with Noninvasive Positive-pressure Ventilation Based on the Prediction of Disease Onset Using CT and Respiratory Function Tests in an Elderly Patient with Relapsing Polychondritis

Article

May 2013
INTERNAL MED

An 83-year-old man who had been receiving treatment for bronchial asthma since 62 years of age experienced difficulty breathing on exertion and was admitted to the hospital. On admission, computed tomography revealed tracheal wall thickening, while test results for antinuclear antibodies and anti-type II collagen antibodies were positive. Since a saddle nose deformity, malacia of the auricles and sensorineural deafness were also observed, relapsing polychondritis was diagnosed. Measuring the peak expiratory flow rate was useful in the early airway assessment. During the follow-up period, the patient's dyspnea worsened and noninvasive positive-pressure ventilation was introduced. As a result, the subjective symptoms improved.

Refractory Relapsing Polychondritis Treated with Serial Success with Interleukin 6 Receptor Blockade

Article

Jan 2013

To the Editor: Relapsing polychondritis (RPC) can affect the ears, eyes, larynx, trachea, bronchi, joints, audiovestibular system, and heart valves1,2,3. Therapy is difficult in the subset of patients refractory to glucocorticoids and conventional immunosuppressive agents. We describe a 65-year-old woman with RPC refractory to glucocorticoids, cyclophosphamide, and infliximab. Interleukin 6 (IL-6) inhibition led to prompt control of RPC affecting her ears, nose, and trachea, as well as swift normalization of her acute-phase reactants. Re-treatment with tocilizumab on 2 occasions following disease flares again led to prompt disease control without the use of other medications. The patient presented with 4 months of intermittent but progressive swelling, erythema, and pain of her right ear (Figure 1A). The nasal bridge was swollen and faintly erythematous (Figure 1B), and the trachea was tender. Her left ear appeared normal (Figure 1C). She had thrombocytosis (platelets 767,000/mm3, normal 150,000−400,000/mm3) and elevations of the erythrocyte sedimentation rate (ESR; 103 mm/h, normal < 17 mm/h) and C-reactive protein (CRP; 170 mg/dl, normal < 8 mg/dl). Given her clinical presentation, a diagnosis of RPC was rendered … Address correspondence to Dr. J.H. Stone, Rheumatology Unit, Massachusetts General Hospital, 55 Fruit St., Yawkey 2, Boston, MA 02114, USA. E-mail: jhstone{at}partners.org

The Relapsing Polychondritis Disease Activity Index: Development of a disease activity score for relapsing polychondritis

Article

Jul 2012

Objective: The rarity of relapsing polychondritis (RP) has hindered the development of standardized tools for clinical assessment. Here, we describe the development of a preliminary score for disease assessing activity in RP, the Relapsing Polychondritis Disease Activity Index (RPDAI). Methods: Twenty-seven RP experts participated in an international collaboration. Selection and definition of items for disease activity were established by consensus during a 4-round internet-based Delphi survey. Twenty-six experts assessed the Physician's Global Assessment (PGA) of disease activity on 43 test cases on a 0-100 scale, yielding a total of 1118 PGA ratings. The weight of each item was estimated by multivariate regression models with generalized estimating equation, using PGA as the dependent variable. Results: Experts decided in consensus that the RPDAI should consider the 28-day period before each RPDAI assessment. Inter-rater reliability assessed by the intra-class correlation coefficient for the 1118 PGA ratings was 0.51 (CI95%: 0.41-0.64). The final RPDAI score comprised 27 items with individual weights ranging from 1 to 24 and a maximum theoretical RPDAI score of 265. Correlation between the RPDAI scores calculated based on the weights derived from the final multivariate model, and the 1118 PGA ratings was good (r=0.56, p<0.0001). Conclusion: We have developed the first consensus scoring system to measure disease activity in relapsing polychondritis (see www.RPDAI.org for online scoring). This tool will be valuable for improving the care of patients with this rare disease.

Erfolgreiche Therapie mit Etanercept bei rezidivierender Polychondritis

Article

Jun 2010

Relapsing polychondritis is a rare autoimmune disease associated with inflammation and destruction of cartilage and connective tissue. We report on a patient with a severe form of this disease that had a progressive and complicated course despite administration of a number of disease-modifying anti-rheumatic drugs. Finally, therapy with the TNF-α-antagonist etanercept was initiated, which led to a considerable decrease in disease activity. This case is further evidence for the efficiency of TNF-α-antagonists in relapsing polychondritis.

Refractory relapsing polychondritis in a child treated with antiCD20 monoclonal antibody (rituximab): First case report

Article

Apr 2012

To report the first case of refractory relapsing polychondritis in a child who was treated with the biological agent, rituximab, an antiCD20 monoclonal antibody. The case is reported with a review of the literature on the use of biological agents in the treatment of refractory relapsing polychondritis. A 10-year-old boy presented with relapsing polychondritis who was treated initially with prednisolone and methotrexate. As there was no response to the treatment, anti TNF antagonist infliximab was given but with a failed response. A subsequent therapy with rituximab produced significant clinical remission with no recurrence at 1 year. Relapsing polychondritis unresponsive to primary treatment modalities but treated with various biological agents in adult have been well described in adults but not reported in children age below 13 yrs. Hence we present this case report. Biological agents such as rituximab has promising role in children when primary treatment fails as reported in our case.

Biologics in Relapsing Polychondritis: A Literature Review

Article

Nov 2011

There is no standardized therapeutic protocol for relapsing polychondritis (RP). Emergence of biologics holds much hope in the management of this connective tissue disease. To evaluate the efficacy and safety of biologics in patients with active RP. A systematic review of the literature using PubMed was performed through December 2010. MeSH terms and keywords were used relating to RP and biologics. All papers reporting the efficacy and/or safety of biologics in RP were selected. Reference lists of included papers were also searched. All publications relate to case series or isolated case reports. No randomized controlled trial has been performed. Thirty papers that included 62 patients were published. These patients were treated with TNFα blockers (n = 43), rituximab (n = 11), anakinra (n = 5), tocilizumab (n = 2), and abatacept (n = 1). The endpoint of treatment differs from 1 publication to the other and therefore makes the comparison of efficacy among the various biologics difficult. Biologics were effective in 27 patients, partially effective in 5 patients, and not effective in 29 patients. Safety appeared to be good. However, 4 deaths were recorded (2 sepsis, 1 postoperatively after aortic aneurysm surgery, and 1 after accidental dislocation of the tracheostomy device). The experience with biologics in RP is very limited and their real efficacy and indications need to be better defined. Randomized controlled trials, although difficult to perform because of the rarity of RP, are needed to determine the place of biologics in the treatment strategy of this orphan disease.

Dementia in a patient with nonparaneoplastic limbic encephalitis associated with relapsing polychondritis [10]

Article

Dec 2006

Infliximab in relapsing polychondritis

Article

Apr 2010

Relapsing polychondritis (RP) is a rare systemic disease of unknown etiology, characterized by recurrent inflammation of cartilaginous structures and other connective tissues, including the ears, nose, joints, respiratory tract, and others. Due to the presence of typical signs and symptoms, biopsy is seldom necessary. Treatment includes corticosteroids, occasionally associated with immunosuppressive agents, but refractory cases are described. Recent reports suggest that anti-TNF agents, such as infliximab, may be of value in patients who do not respond to conventional therapy, but experience with this treatment is scarce. In this paper, the authors report the case of a patient with RP refractory to combined treatment with corticosteroids and immunosuppressive agents, who showed a good response to infliximab.

Pulmonary Manifestations of Relapsing Polychondritis

Article

Sep 2010

Relapsing polychondritis (RP) is a chronic multisystemic disease characterized by recurrent episodes of cartilage inflammation throughout the body. The lower respiratory tract is involved in 20% to 50% of patients and results in significant morbidity. Effective medical therapies and airway interventions are available in experienced centers; however, no single treatment is curative, and the prognosis of RP with airway disease remains overall guarded.

Relapsing polychondritis: An autoimmune disease with many faces

Article

Mar 2010

Relapsing polychondritis (RPC) is a rare immune mediated disease which is associated with inflammation in cartilaginous tissue throughout the body. Especially the cartilaginous structures of ear, nose, joints and respiratory tract are affected. In around 30% of the cases an association with other diseases especially systemic vasculitis or myelodysplatic syndrome can be detected. The relative rarity of RPC has not permitted clinical trials to determine the efficacy and safety of therapy strategies. Often the medication in current use is largely empiric and based on case reports. Therefore different immunosuppressants such as cyclophosphamide, azathioprine, cyclosporine, mycophenolate mofetil and also new approaches like tumor necrosis factor alpha blockers (TNF-alpha antagonists) have been used for the treatment of severe manifestations of RPC with varying degrees of efficacy. This review gives a close look to clinical manifestation, diagnosis and also therapy options of RPC.

[Successful therapy with etanercept in relapsing polychondritis].

Article

Jun 2010
Z RHEUMATOL

Relapsing polychondritis is a rare autoimmune disease associated with inflammation and destruction of cartilage and connective tissue.We report on a patient with a severe form of this disease that had a progressive and complicated course despite administration of a number of disease-modifying anti-rheumatic drugs.Finally, therapy with the TNF-alpha-antagonist etanercept was initiated, which led to a considerable decrease in disease activity. This case is further evidence for the efficiency of TNF-alpha-antagonists in relapsing polychondritis.

Relapsing polychondritis: New therapeutic strategies with biological agents

Article

Sep 2009
RHEUMATOL INT

Relapsing polychondritis (RP) is a rare disease of unknown etiology characterized by recurrent episodes of inflammation resulting in the destruction of cartilaginous tissues. We describe a young girl with RP unresponsive to conventional therapy.

Sustained response to infliximab in a patient with relapsing polychondritis with aortic involvement

Article

Sep 2009

Takayasu disease presenting as malignant pyoderma gangrenosum in a child with relapsing polychondritis

Article

Dec 2008

A 7-year-old Lebanese girl with recently diagnosed relapsing polychondritis had a 1-month history of several painful ulcerations involving her entire body. Skin biopsy was consistent with the diagnosis of pyoderma gangrenosum. She was hospitalized and started on intravenous steroids with partial improvement. During her hospital stay, she developed right wrist drop. Radiologic studies revealed a large aortic aneurysm and an axillary aneurysm compressing the right brachial nerve plexus. Pathology of the resected aortic anyeurism confirmed the diagnosis of Takayasu's arteritis. The patient was started on infliximab therapy with complete resolution of her skin lesions and improvement in hand function.

Relapsing Polychondritis and Airway Involvement

Article

Dec 2008
CHEST

To assess the prevalence and characteristics of airway involvement in relapsing polychondritis (RP). Retrospective chart review and data analysis of RP patients seen in the Rheumatology Clinic and the Complex Airway Center at Beth Israel Deaconess Medical Center from January 2004 through February 2008. RP was diagnosed in 145 patients. Thirty-one patients had airway involvement, a prevalence of 21%. Twenty-two patients were women (70%), and they were between 11 and 61 years of age (median age, 42 years) at the time of first symptoms. Airway symptoms were the first manifestation of disease in 17 patients (54%). Dyspnea was the most common symptom in 20 patients (64%), followed by cough, stridor, and hoarseness. Airway problems included the following: subglottic stenosis (n = 8; 26%); focal and diffuse malacia (n = 15; 48%); and focal stenosis in different areas of the bronchial tree in the rest of the patients. Twelve patients (40%) required and underwent intervention including balloon dilatation, stent placement, tracheotomy, or a combination of the above with good success. The majority of patients experienced improvement in airway symptoms after intervention. One patient died during the follow-up period from the progression of airway disease. The rest of the patients continue to undergo periodic evaluation and intervention. In this largest cohort described in the English language literature, we found symptomatic airway involvement in RP to be common and at times severe. The nature of airway problems is diverse, with tracheomalacia being the most common. Airway intervention is frequently required and in experienced hands results in symptom improvement.

Severe respiratory dysfunction in a patient with relapsing polychondritis: should we treat the saddle nose deformity?

Article

Nov 2008

We report a case of surgical correction of a saddle nose deformity, causing severe ventilation restrictions in a 42-year-old man diagnosed with relapsing polychondritis. Relapsing polychondritis is an autoimmune disorder, in which antibodies to type II collagen cause an inflammatory destruction of cartilage. If septal cartilage of the nose is involved, destruction leads to collapse of the dorsum of the nose, causing a saddle nose deformity. Patients suffer from a ventilation disorder of varying degree depending on the response to or onset of immunosuppressive therapy. In the described patient, the destruction of the nasal septum, in addition to unstable tracheal cartilage, caused a severe restriction in ventilation, with total collapse of the internal nasal valves during forced inspiration. To improve the function of the external airways the patient underwent surgery to reconstruct the nasal septum. Although cartilage grafts are the state of the art to reconstruct the nasal septum, we used a bone graft from the iliac crest, because the autoimmune polychondritis precludes cartilage grafting due to expected cartilage destruction. At follow up 2 years postoperatively no signs of bone resorption or deterioration of the improved airway were observed. We conclude that the use of bone grafts is a promising method to restore and improve ventilation disorders caused by a saddle nose deformity in relapsing polychondritis.

Successful treatment of recalcitrant relapsing polychondritis with monoclonal antibodies

Article

Sep 2008
J EUR ACAD DERMATOL

Relapsing polychondritis: Prospective study of 23 patients and a review of the literature

Article

Jun 1976

Relapsing polychondritis (RP) is not a totally rare rheumatic disease. We have seen 23 patients from 1960-1975, and there are now a total of 159 reported cases, which form the basis of this study. RP occurs equally in both sexes, and has a maximum frequency in the fourth decade. 2) Empirically defined diagnostic criteria are proposed, to include the most common clinical features: a) Bilateral auricular chondritis b) Nonerosive sero-negative inflammatory polyarthritis c) nasal chondritis d) Ocular inflammation e) Respiratory tract chondritis f) Audiovestibular damage The diagnosis is based primarly upon the unique clinical features, and is quite certain if three or more criteria are present together with histologic confirmation. 3) Fifty percent of patients present with either auricular chondritis or the arthropathy of RP; but with prolonged follow-up, a majority of patients develop four or more of the above mentioned criteria. 4) Approximately 30 percent of patients have a preceding or coexistent rheumatic or autoimmune disease, which can lead to initial diagnostic confusion. 5) Laboratory and radiographic investigations help mainly to rule out other diagnostic possibilities, with no characteristic abnormalities being present in a majority of patients. 6) On follow-up, three-fourths of our patients required chronic corticosteroid therapy with an average dose of 25 mg per day of prednisone. Corticosteroids decrease the frequency, duration, and severity of flares, but do not stop disease progression in severe cases. 7) The mortality rate has been 30 percent in our series and 22 percent in the other 136 reported cases. Of the 29 cases where the cause of death was known, 17 were from respiratory tract involvement and 9 from cardiac valvular or vasculitic involvement, emphasizing the need to search for critical involvement of either of these organ systems in each patient. 8) Detailed reports of selected cases are presented to illustrate the clinical diagnosis and differential diagnosis, and to demonstrate the need for careful prolonged follow-up. 9) Although the etiology remains unknown, there is a frequent association with, and clinical similarity to, other rheumatic diseases. 10) Careful clinicopathological study of our 23 patients leads us to postulate an underying systemic vascultis as an important pathologic mechanism in RP.

Vascular involvement in relapsing polychondritis

Article

Jun 1977
Can Med Assoc J

Review of four cases of relapsing polychondritis (RP) seen at one hospital in the 12-year period 1963 to 1974 revealed that one patient had aortic insufficiency with large artery involvement, two others had involvement of medium and large arteries and the fourth may have had mucocutaneous vasculitis. Valvular disease has occurred in 9% of all cases of RP reported in the literature and, if vasculitis beyong the aortic root is included, 25% of cases of RP manifested inflammatory vascular disease. The frequency of pseudotumour of the orbit and cochlear-labyrinthine dysfunction is also high and may be a manifestation of vasculitis.

Relapsing polychondritis: Survival and predictive role of early disease manifestations

Article

Feb 1986

To define the natural history of relapsing polychondritis, the probability of survival and causes of death were determined in 112 patients seen at one institution. By using covariate analysis, early clinical manifestations were identified that predicted mortality. The 5- and 10-year probabilities of survival after diagnosis were 74% and 55%, respectively. The most frequent causes of death were infection, systemic vasculitis, and malignancy. Only 10% of the deaths could be attributed to airway involvement by chondritis. Anemia at diagnosis was a marker for decreased survival in the entire group. There was an interaction between other disease variables and age in determining their impact on outcome. For patients less than 51 years old, saddle-nose deformity and systemic vasculitis were the worst prognostic signs. For older patients, only anemia predicted outcome. The need for corticosteroid therapy did not influence survival.

Ocular and Systemic Findings in Relapsing Polychondritis

Article

Jun 1986
OPHTHALMOLOGY

The ocular and systemic findings in 112 Mayo Clinic patients with relapsing polychondritis were reviewed. The incidence of males and females was equal, with median age at diagnosis of 51 years and the median follow-up of 6 years. Most patients had several organ systems involved at the time of the diagnosis. Twenty-one patients had ocular symptoms at the onset, and 57 developed ocular symptoms during their course. Major ocular complications included proptosis, lid edema, episcleritis/scleritis, corneal infiltrates/thinning, iridocyclitis, retinopathy, and optic neuritis. The major system involvement included otorhinolaryngeal, respiratory, arthritic, renal, cardiovascular, dermatologic, and neurologic diseases. Generally, laboratory studies were not helpful in making the diagnosis but were valuable in monitoring the disease. Based on the experience in these cases, the indications for the various therapeutic modalities are offered.

Renal Involvement in Relapsing Polychondritis

Article

Jun 1987

Twenty-nine of the 129 patients with RP seen at the Mayo Clinic between 1943 and 1984 had renal involvement. These patients were older, had arthritis and extrarenal vasculitis more frequently, and had a significantly worse survival rate than those without renal involvement. Renal biopsies were obtained in 11 of these 29 patients. The predominant lesions were mild mesangial expansion and cell proliferation, and segmental necrotizing glomerulonephritis with crescents. Small amounts of electron-dense deposits, predominantly mesangial, were noted on electron microscopy. Immunofluorescence revealed faint deposition of C3 and/or IgG or IgM, predominantly in the mesangium. Autopsies were obtained in 13 of the 47 patients who had died. Information regarding the renal pathology was available in 10 of these 13 autopsies. At the time of the initial evaluation at the Mayo Clinic, 6 of these 10 patients had evidence of renal involvement. At autopsy, none of these 10 patients had evidence of active renal vasculitis or segmental necrotizing glomerulonephritis, but 8 of the 10 patients exhibited variable degrees of vascular and glomerular sclerosis, segmental mesangial proliferation, tubular loss, and interstitial lymphocytic infiltrates. These observations expand the limited information available in the literature, which is based on 11 previously published case reports of renal involvement in RP. In only a few of our patients and previously reported patients were the manifestations of the disease limited to the systems characteristically involved in pure RP. The frequent coexistence of other autoimmune and connective tissue diseases supports the role of immune mechanisms in the pathogenesis of this syndrome. Deposition of immune complexes is likely to play a role in the pathogenesis of the glomerular lesions associated with RP. Administration of corticosteroids alone is sufficient to induce a complete remission in some cases, while in others the addition of a cytotoxic agent is necessary to control the activity of the disease or to spare corticosteroid side effects and maintain a remission. Immunosuppression-related infectious complications and undetected relapses after discontinuation of immunosuppressive therapy are largely responsible for the morbidity and mortality observed in these patients.

Mouth and genital ulcers with inflamed cartilage: MAGIC syndrome. Five patients with features of relapsing polychondritis and Behçet's disease

Article

Aug 1985
AM J MED

Five patients with features of coexistent relapsing polychondritis and Behçet's disease are described. Review of the literature supports the overlap of the clinical manifestations of these two conditions. A common immunologic abnormality is likely, and elastin is cited as a possible target antigen. The "mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome" is the proposed name for this entity.

Cardiac valve replacement in relapsing polychondritis. A review

Article

Jun 1995
J HEART VALVE DIS

Cardiac valve replacement is a rare but not exceptional eventuality in patients with relapsing polychondritis. One case requiring aortic and mitral valve replacement and its follow up is described. From the review of the literature an additional twenty patients who required cardiac valve replacement are analyzed. The mean delay between the first onset of relapsing polychondritis and operation was 6.51 years and the mean age at operation was 38.8 years. There was a preponderance of male patients (73.7%). Aortic and mitral valves were replaced in 100% and 28.5% of patients, respectively. During the four first postoperative years 23.8% of them were reoperated for periprosthetic leak or aortic aneurysm, and during the same period 52.6% died of a cardiovascular cause. Immunosuppressive agents should be employed in patients with relapsing polychondritis and cardiovascular involvement because they seem to be more effective than steroids in severe forms of the disease. Therefore, we recommend close and prolonged follow up: firstly because there can be early paravalvular prosthetic leakage due to the friability of the tissue to which it has been anchored; secondly because aortic aneurysms occur frequently in relapsing polychondritis, may be multiple, may involve all parts of the aorta and result in fatal rupture even in asymptomatic patients; and thirdly because there can be a fatal outcome due to other organ involvement, like airway obstruction, acute glomerulonephritis, or systemic vasculitis. Prophylactic composite graft replacement of the ascending aorta associated with replacement of the aortic valve and re-implantation of the coronary arteries could avoid the need for reoperation in these high risk patients.

Nasal Continuous Positive Airway Pressure in the Treatment of Tracheobronchomalacia

Article

Mar 1993
Am J Respir Crit Care Med

The effect of nasal continuous positive airway pressure (CPAP) was assessed in three patients with tracheobronchomalacia (TBM) who failed conventional medical management. Using physiologic measures of airflow and fiberoptic bronchoscopy, we evaluated expiratory airflow and airway collapse during the acute administration of nasal CPAP. FVC increased and dynamic airway collapse [slow vital capacity minus forced vital capacity (SVC--FVC)] decreased with increasing levels of CPAP. Notching associated with airway collapse was observed in the baseline spirograms of all three patients, and it disappeared with the addition of nasal CPAP. Fiberoptic bronchoscopy confirmed the severity of TBM in each patient and documented an acute improvement in expiratory airway collapse with the addition of nasal CPAP. Intermittent nasal CPAP was then added to the patients' treatment regimens, improving the course of their disease. Specific treatment outcomes varied from patient to patient, but they included improved sputum production, atelectasis, exercise tolerance, and patient symptoms plus reduced need for medical care. These findings suggest that the addition of intermittent nasal CPAP to routine medical therapy may be of benefit to patients with severe TBM unresponsive to conventional medical management.

Cardiovascular involvement in relapsing polychondritis

Article

Jul 1997
SEMIN ARTHRITIS RHEU

Attilio Del Rosso

Relapsing polychondritis: a clinical review for rheumatologists

Abstract

No full-text available