No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
Patient-Perception of Disease-Related Symptoms and Complications in Relapsing Polychondritis
Arthritis Care & Research
Abstract
Objective:
To assess patient-reported symptoms and burden of disease in relapsing polychondritis (RP).
Methods:
Patients with RP completed a disease-specific online survey to identify symptoms attributed to illness. Patients were divided into subgroups based upon presence or absence of ear/nose, airway, or joint involvement. Pathway to diagnosis, treatment, and disease-related complications were assessed within each subgroup.
Results:
Data from 304 respondents were included in this analysis. Prior to diagnosis, most patients with RP went to the emergency room (54%), saw >3 physicians (54%), and had symptoms for >5 years (64%). A concomitant diagnosis of fibromyalgia and absence of ear/nose or joint involvement was associated with diagnostic delay >1 year. Common diagnoses prior to RP diagnosis included asthma in patients with airway involvement (35% vs 22%, p=0.03) and ear infection in patients with ear/nose involvement (51% vs 6%, p<0.01). Patients with joint involvement were more likely to receive a glucocorticoid-sparing agent (85% vs 13%, p<0.01). Most patients reported a major complication including disability (25%), tracheomalacia (16%) or hearing loss (34%). Patients with airway involvement reported more tracheomalacia (20% vs 4%, p<0.01). Disability (24% vs 7%, p<0.01) and hearing loss (39% vs 11%, p<0.01) were prevalent in the joint involvement subgroup.
Conclusion:
Patient-reported data in RP highlight a significant burden of disease. Patterns of organ involvement may lead to diagnostic delay and influence treatment decisions, ultimately impacting the development of disease-related complications. Timely diagnosis, standardization of treatment approaches, and prevention of disease-related complications are major unmet needs in RP. This article is protected by copyright. All rights reserved.
... Patients with laryngo-tracheal involvement or auricular in ammation as the only initial feature of the disease [13], sometimes as the sole feature [8,[17][18][19][20][21][22][23][24], have been de ned as limited form of RPC. In our cohort, 52.3% of the patients presented as limited RPC initially and 40.5% of them remained limited form during follow-up, suggesting that limited RPC is not uncommon among Chinese patients. ...
... In our cohort, 52.3% of the patients presented as limited RPC initially and 40.5% of them remained limited form during follow-up, suggesting that limited RPC is not uncommon among Chinese patients. The concept of limited and systemic form is also supported by a recent online survey in the US [8], but the incidence of limited RPC was much lower (16 and 2 out of 304 patients presented as isolated ear involvement and isolated airway involvement respectively). ...
... Patients that underwent recurrent chondritis associated with deformity, vestibular dysfunction, ocular in ammation, or in ammatory arthritis were diagnosed as partial RPC as suggested by Mathew et al. [11]. Limited RPC can also be diagnosed if patients presented with recurrent in ammatory episodes at isolated cartilaginous sites after exclusion of other possible causes and are responsive to glucocorticoids, based on the observations by other groups and our own [6,8,13,[17][18][19][20][21][22][23][24] . The diagnostic criteria used in present study were listed in table 4. Patients younger than 18 years or without complete electronic case les were excluded. ...
Background Relapsing polychondritis(RPC) is a rare autoimmune disease and its early diagnosis pose a challenge to medical committee. 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. Their average age at onset were 47.1±13.8 years with a median follow-up of 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, some patients did become systemic form. Patients with Ear pattern had the highest survival rate and 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, and clinical patterns and presenting forms can evolve during follow-up. Our findings may facilitate early recognition of this rare disease.
... Early identification of RP, with prompt initiation of treat ment, may help decrease uncontrolled inflammation and ultimate organ damage. RP carries significant morbidity, including tracheo malacia, subglottic stenosis, intensive care unit (ICU) admissions, hearing loss, and disability (3,4). Significant mortality is also asso ciated with RP, with an incidence rate ranging from 55% to 91% 10 years after diagnosis (3,5). ...
... Significant mortality is also asso ciated with RP, with an incidence rate ranging from 55% to 91% 10 years after diagnosis (3,5). Diagnostic delay is common, with reported time to diagnosis ranging anywhere from 1 year to 20 years (4,6). Lack of auricular chondritis has been associated with diagnostic delay, indicating that broader understanding of the spectrum of possible clinical manifestations and patterns of organ involvement could facilitate a more timely diagnosis (4). ...
... Diagnostic delay is common, with reported time to diagnosis ranging anywhere from 1 year to 20 years (4,6). Lack of auricular chondritis has been associated with diagnostic delay, indicating that broader understanding of the spectrum of possible clinical manifestations and patterns of organ involvement could facilitate a more timely diagnosis (4). ...
Objective
Relapsing polychondritis (RP) is a systemic disease. Failure to recognize RP can lead to diagnostic delay and further complications, including death. This study was undertaken to identify clinical patterns in a prospective cohort of patients with RP.
Methods
Patient subgroups were identified using latent class analysis based on 8 clinical variables: saddle‐nose deformity, subglottic stenosis, tracheomalacia, bronchomalacia, ear chondritis, tenosynovitis/synovitis, inflammatory eye disease, and audiovestibular disease. Model selection was based on Akaike's information criterion.
Results
Seventy‐three patients were included in this study. Patients were classified into 1 of 3 subgroups: type 1 RP (14%), type 2 RP (29%), and type 3 RP (58%). Type 1 RP was characterized by ear chondritis (100%), tracheomalacia (100%), saddle‐nose deformity (90%), and subglottic stenosis (80%). These patients had the shortest median time to diagnosis (1 year), highest disease activity, and greatest frequency of admission to the intensive care unit and tracheostomy. Type 2 RP was characterized by tracheomalacia (100%) and bronchomalacia (52%), but no saddle‐nose deformity or subglottic stenosis. These patients had the longest median time to diagnosis (10 years) and highest percentage of work disability. Type 3 RP was characterized by tenosynovitis/synovitis (60%) and ear chondritis (55%). There were no significant differences in sex, race, or treatment strategies between the 3 subgroups.
Conclusion
Our findings indicate that there are 3 subgroups of patients with RP, with differences in time to diagnosis, clinical and radiologic characteristics, and disease‐related complications. Recognizing a broader spectrum of clinical patterns in RP, beyond cartilaginous involvement of the ear and upper airway, may facilitate more timely diagnosis.
... Beyond these categories, patients with other systemic autoimmune conditions can develop vasculitis as an associated disease feature. For example, relapsing polychondritis (RP) is a rare disease characterized by inflammation of cartilaginous structures, with associated vasculitis reported in 10-20% of these patients (2,3). The various forms of vasculitis are generally chronic, unpredictable, relapsing illnesses that pose significant physical and psychological burdens on patients (2,4). ...
... For example, relapsing polychondritis (RP) is a rare disease characterized by inflammation of cartilaginous structures, with associated vasculitis reported in 10-20% of these patients (2,3). The various forms of vasculitis are generally chronic, unpredictable, relapsing illnesses that pose significant physical and psychological burdens on patients (2,4). ...
... Further, this study uniquely included data from patients with RP, and demonstrated a high level of patientperceived burden of illness in these patients (2). Finally, multiple validated questionnaires were administered concurrently within a standardized protocol to comprehensively assess the relationship between illness perceptions and other measures of disease activity in vasculitis. ...
Objective
To assess the validity and clinical utility of the Brief Illness Perception Questionnaire (BIPQ) to measure illness perceptions in multiple forms of vasculitis.
Methods
Patients with giant cell arteritis (GCA), Takayasu’s arteritis (TAK), ANCA-associated vasculitis (AAV) and relapsing polychondritis (RP) were recruited into a prospective, observational cohort. Patients independently completed the BIPQ, Multidimensional Fatigue Inventory (MFI), SF-36 Health Survey (SF-36) and a Patient Global Assessment (PtGA) at successive study visits. Physicians concurrently completed a Physician Global Assessment (PhGA) form. Illness perceptions, as assessed by the BIPQ, were compared to responses from the full-length Revised Illness Perception Questionnaire (IPQ-R) and to other clinical outcome measures.
Results
196 patients (GCA=47, TAK=47, RP=56, AAV=46) were evaluated over 454 visits. Illness perception scores in each domain were comparable between the BIPQ and IPQ-R (3.28 vs 3.47, p=0.22). Illness perceptions differed by type of vasculitis, with the highest perceived psychological burden of disease in RP. The BIPQ was significantly associated with all other patient-reported outcome measures (ρ=0.50-0.70, p<0.0001) but did not correlate with PhGA (ρ=0.13, p=0.13). A change in the BIPQ composite score of ≥7 over successive visits was associated with concomitant change in the PtGA. Change in the MFI and BIPQ scores significantly correlated over time (ρ= 0.38, p=0.0008).
Conclusion
The BIPQ is an accurate and valid assessment tool to measure and monitor illness perceptions in patients with vasculitis. Use of the BIPQ as an outcome measure in clinical trials may provide complimentary information to physician-based assessments.
... million population per year between 1990 and 2012 in the UK [5]. Patients may visit physicians at different departments, mainly non-rheumatologic specialists [5][6][7][8], due to protean manifestations and wax and wane disease courses of RPC, and often experience a misdiagnosis [6][7][8], leading to delayed diagnosis [5][6][7][8][9][10][11][12][13]. ...
... million population per year between 1990 and 2012 in the UK [5]. Patients may visit physicians at different departments, mainly non-rheumatologic specialists [5][6][7][8], due to protean manifestations and wax and wane disease courses of RPC, and often experience a misdiagnosis [6][7][8], leading to delayed diagnosis [5][6][7][8][9][10][11][12][13]. ...
... million population per year between 1990 and 2012 in the UK [5]. Patients may visit physicians at different departments, mainly non-rheumatologic specialists [5][6][7][8], due to protean manifestations and wax and wane disease courses of RPC, and often experience a misdiagnosis [6][7][8], leading to delayed diagnosis [5][6][7][8][9][10][11][12][13]. ...
Objective To explore the clinical patterns of relapsing polychondritis(RPC) to improve clinicians’ comprehensive understanding of the disease.
Methods The electronic records of RPC patients that were hospitalized and followed-up by rheumatologists at our hospital between January 1, 2008 and August 31,2019 were retrospectively analyzed.
Results Sixty-six males and 60 femaleswere included with onset age of 47.1±13.8 years and followed up for a median of 18 months. Eighty-seven patients (69.1%) did not met with the traditional criteria.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 subdivided as limited and systemic form of RPC (27.8%with limited form of Ear pattern and 24.6% with limited form of Airway pattern). During follow-up, a small part of patients with Ear pattern and Airway pattern progressed into Overlap pattern andsome Airway-Ear negative patternpatients progressed into Ear pattern. Most of the limited RPC patientsremained limited form during follow-up whilesome patients with limited form progressed into systemic form. Patients with Ear pattern had the highest survival rate and relative lower inflammatory status.
Conclusion RPC patients can be categorized as 4 differentclinical patterns and 2 distinct presenting forms (limited and systemic) based on organ involvement.Limited form of RPC is not uncommon in Chinese patients and traditional criteria of RPC are not suitable for early diagnosis.
... 1,3 Due to the frequent spontaneous resolution of flares and variability of presentation, the diagnosis of relapsing polychondritis is often delayed for years after initial symptom onset. 3,4 Auricular chondritis affects approximately 90% of patients with relapsing polychondritis. 1 Recurrent episodes can destroy auricular cartilage, resulting in a cauliflower appearance of the ear. Hearing loss occurs in approximately 40% of patients with relapsing polychondritis. ...
... 6 Asymmetric, nonerosive arthritis of large and small joints occurs in approximately half of patients with relapsing polychondritis, and many experience systemic symptoms such as fatigue and weight loss. 1,3,4,6 Because no validated diagnostic criteria exist, the diagnosis of relapsing polychondritis is primarily based on symptoms and physical examination findings consistent with chondritis. 1,2 There are no specific laboratory tests for diagnosis; however, C-reactive protein and ESR are elevated in approximately 60% of patients. ...
... Relapsing polychondritis (RP) is a rare, systemic, chronic, disabling condition that primarily affects cartilage [1]. RP can involve multiple organs, including the musculoskeletal system, airway, ear, nose, eyes, skin, vasculature and neurological system, resulting in a high disease burden [2]. Patient-reported outcome measures (PROMs) demonstrate how disease manifestations affect patients and provide unique insight about the impact of a disease [3][4][5]. ...
... Patients with RP report a high psychological burden of disease, exposing a need for patient-centred approaches to address pain, fatigue and the emotional impact of illness. In addition to treatment of underlying inflammation, therapeutic approaches that address the psychological burden of disease are advisable, including interventions that improve physical and mental health [2,32]. The use of multifaceted treatment approaches that address the physical and psychological burden of disease in RP may lead to greater improvement in a patient's functional status, quality of life and selfreported disease assessment. ...
Objectives
Relapsing polychondritis (RP) is a rare, heterogeneous, systemic inflammatory disease that targets cartilage. Patient-reported outcome measures may differ from physician assessment. This study compared patient global assessment (PtGA) and physician global assessment (PhGA) scores in a prospective cohort of patients with RP.
Methods
Adult patients with RP underwent a standardized comprehensive evaluation at ∼6-month intervals. At each visit, three physicians scored PhGA by consensus, and the patient independently completed four patient-reported outcomes: PtGA, SF-36 Health Survey, Brief Illness Perception Questionnaire (BIPQ), and Multidimensional Fatigue Inventory (MFI). Patient—physician discordance was defined as a difference between PtGA and PhGA of ≥ 3 on a 0–10 scale.
Results
76 patients were evaluated over 154 visits. Median PhGA was 3 (interquartile range 2–3), and median PtGA was 5 (interquartile range 4–7). PtGA and PhGA were concordant in 66 visits (42.9%), and patients scored disease severity ≥3 points higher than physicians scored disease activity (positive discordance) in 84 visits (54.5%). Compared with visits with concordance, visits with positive discordance were associated with significantly worse scores on the MFI, BIPQ, SF-36 Physical component score (PCS), and SF-36 Mental component score (MCS).
Conclusion
Patients with RP typically self-report high PtGA which does not align with PhGA. Discordance is likely driven by the high physical and psychological burden of illness experienced by patients. Multifaceted treatment approaches that address the burden of disease in RP from the patient-perspective are needed.
... The diagnostic delay is associated with the lack of ear, nose or joint involvement. [10][11][12][13] Early signs of RP in such cases may be intermittent arthritis or eye involvement such as episcleritis and scleritis, which may point a search for RP. However, even in the presence of external ear inflammation, an infection, local trauma, insect bite and chondrodermatitis nodularis helicis (Winkler's disease) are often suspected first. ...
... Mortality in RP is more than double compared with the general population; the most frequent causes of death are respiratory, cardiac and haematological involvement. 12 Three phenotypes with different presentations have been recently described in a cluster analysis of a cohort: a haematological form (in 10% of cases), a respiratory form (in 25%) and a mild one with good prognosis (about 65% of cases). 15 Some complications such as aortitis, 18 vasculitis 4 or anaemia are pejorative prognosis factors. ...
Due to the rarity of relapsing polychondritis (RP), many unmet needs remain in the management of RP. Here, we present a systematic review of clinical practice guidelines (CPGs) published for RP, as well as a list of the most striking unmet needs for this rare disease. We carried out a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) and keywords of the disease and publication type (CPGs). The systematic literature review identified 20 citations, among which no CPGs could be identified. We identified 11 main areas with unmet needs in the field of RP: the diagnosis strategy for RP; the therapeutic management of RP; the management of pregnancy in RP; the management of the disease in specific age groups (for instance in paediatric-onset RP); the evaluation of adherence to treatment; the follow-up of patients with RP, including the frequency of screening for the potential complications and the optimal imaging tools for each involved region; perioperative and anaesthetic management (due to tracheal involvement); risk of neoplasms in RP, including haematological malignancies; the prevention and management of infections; tools for assessment of disease activity and damage; and patient-reported outcomes and quality of life indicators. Patients and physicians should work together within the frame of the ReCONNET network to derive valuable evidence for obtaining literature-informed CPGs.
... Patients presenting with RP associated with dyspnoea may initially be diagnosed with asthma because they respond to short courses of steroids [8]. However, RP has a progressive course with relapses that worsen pulmonary function with laryngotracheal involvement, which increases morbidity and mortality, as in the case of our patient, who had been a tracheostomy user for 14 years, with multiple episodes of respiratory tract infection (pneumonia, tracheitis) [9]. Therefore, in patients with persistent symptoms of fever and dyspnoea, associated with multiple respiratory infections, without abnormalities in initial chest scans, RP should be considered among the differential diagnoses, because shortening the diagnosis time and early initiation of treatment avoids disease progression [10]. ...
... Absence of ear, nose, or joint involvement, and fibromyalgia were significantly associated with a diagnostic delay >1 year from symptom onset. 28 Unsurprisingly, one study noted that rheumatologists were most frequently responsible for making the final diagnosis of RP. 29 ...
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.
... All patients (n = 72) other studies [4,7,[28][29][30], involvements of skin (5.6%: 13-46%) and cardiovascular involvements (5.6%: 2-31%) were less common. Chang-Miller et al. [31] reported renal involvement in 29 of 129 patients. ...
Background
Relapsing polychondritis (RP) patients with tracheal cartilage involvement are different from other patients. The objective of this study was to compare the clinical features and disease patterns between a respiratory involvement subgroup and a non-respiratory involvement subgroup according to chest computed tomography.
Method
We performed a retrospective cohort study collecting RP patients hospitalized at the Beijing Chao-Yang Hospital between January 2012 and August 2021.
Results
Respiratory involvement affected 59.7% of patients in our cohort. The incidence of costochondritis was more common in RP patients with respiratory involvement ( p = 0.03); the incidence of inflammatory eye disease ( p = 0.001) and auricular chondritis ( p = 0.001) was less frequent in RP respiratory involvement patients.. Compared with the non-respiratory involvement subgroup the incidence of pulmonary infection marginally increased in the respiratory involvement subgroup ( p = 0.06). Inflammatory indexes except for C-reactive protein to albumin ratio (CAR) were significantly higher in the respiratory involvement subgroup; analysis revealed no significant relationship between inflammatory indexes and pulmonary infection.
Conclusion
RP patients with respiratory involvement had a greater incidence of costochondritis and pulmonary infectionand lesser incidence of inflammatory eye diseases and auricular chondritis compared to non-respiratory involvement. Increasing inflammatory indexes suggests that patients with respiratory involvement had a higher disease activity index of RP. The difference in probability of survival was insignificant between subgroups.
... Vasculitis, aortitis, anemia, male gender, uveitis, hearing loss, vestibular disorder, and the need for MP and cyclophosphamide infusion are criteria for a poor prognosis. [9] Conclusion RP is an uncommon, but progressive autoimmune disease which can lead to fatal complications. As in the current patient, especially in females, there may be presentation with saddle nose deformity and it must be emphasized that this should be kept in mind in the differential diagnosis. ...
Relapsing polychondritis (RP) is an uncommon autoimmune disease characterized by repeated and progressive inflammation in cartilage tissue. The auricular cartilage is the location of the most common clinical findings and the most common site of initial involvement. Nasal chondritis is a manifestation present in 15% of patients. Progressive destruction of nasal cartilage leads to the characteristic flattening of the nasal bridge, resulting in saddle nose deformity. The case is, here, presented of a 38-year-old patient who developed nasal chondritis which resulted in saddle nose deformity, and was diagnosed with RP following rhinoplasty.
... We didn't do a further subgroup analysis on account of small quantity, those patients may require to be isolated and analyzed as well as the Overlap subgroup [19]. In contrast to other studies [4,7,[23][24][25], involvements of skin (5.6%: 13-46%) and cardiovascular involvements (5.6%: 2-31%) were less common. Chang-Miller et al [26] reported renal involvement was observed in 29 of 129 patients, however, renal involvement were not present in our study, but uric acid and creatinine levels were higher in the non-respiratory involvement subgroup, reminding possible kidney damage. ...
Background
Relapsing polychondritis (RP) patients with tracheal cartilage involvement are different from other patients. The objectives of this study were to compare the clinical features and disease patterns between respiratory involvement subgroup and non-respiratory involvement subgroup according to chest computed tomography.
Method
We performed a retrospective cohort study collected RP patients hospitalized at the Beijing Chao-Yang Hospital between January 2012 - August 2021.
Results
The incident of costochondritis was more frequent in RP patients with respiratory involvement(p=0.03), the incidence of inflammatory eye disease(p=0.001) and auricular chondritis(p=0.001) was less frequent in RP respiratory involvement patients, compared with those of RP patients without respiratory involvement. Correlation analysis showed that a negative correlation between respiratory involvement and auricular chondritis (r=-0.58, p < 0.01), and between respiratory involvement and inflammatory eye disease (r=-0.45, P < 0.01). Auricular chondritis was positively correlated with inflammatory eye disease (r=0.49, P < 0.01). Compared with non-respiratory involvement subgroup, the incidence of pulmonary infection marginally increased in respiratory involvement subgroup(p=0.06). Inflammatory indexes except for CAR were significantly higher in respiratory involvement subgroup, subgroup analysis found that there was no significant relationship between inflammatory indexes and pulmonary infection.
Conclusion
RP patients with respiratory involvement was characterized by higher rate of costochondritis and pulmonary infection, fewer inflammatory eye disease and auricular chondritis compared to non-respiratory involvement. Increase inflammatory indexes may suggested that patients with respiratory involvement had a higher disease activity index of RP. The probability of survival was not significant between two subgroups.
... Relapsing polychondritis (RP) is a rare connective tissue disease involving multiple organs. 1 A research from UK found that, the incidence of RP between 1990 and 2012 was 0.71 per million population per year. 2 Another study revealed that there was a significant burden of disease on RP patients by analyzing patient-reported data. 3 Horvath et al. 4 conducted a epidemiology study of RP in Hungary, and demonstrated that the good survival rate of RP was possibly related with early diagnosis of the disease. The involvement of the central nervous system (CNS) in RP has occasionally been reported. ...
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.
... Miscellaneous disorders without chondritis may be challenging to diagnose in patients with RP. Absence of ear and nasal involvement was reported to lead to the diagnostic delay [21]. ...
Background:
In patients with relapsing polychondritis (RP), organ involvement developed in those with progressive and/or long disease courses. For their management, elucidation of a subgroup suggesting disease progression is awaited.
Methods:
We previously conducted a physician's questionnaire-based retrospective study to elucidate major clinical features of Japanese patients with RP. We here evaluated organ involvement at disease onset and at the last follow-up. We then counted cumulative numbers of involved organs at the last follow-up in 229 RP patients and compared them with involved organ numbers at disease onset, as possible indicators of disease progression. We assigned their prognosis at the last follow-up into "patient prognostic stages" from no medication (stage 1) to death (stage 5). We utilized nonparametric tests for group comparisons.
Results:
Involved organ numbers per-patient were 1.13 ± 0.03 at disease onset and 3.25 ± 0.10 at the last follow-up (disease duration was 4.69 ± 0.33 years), and increased along with the patient prognostic stages.At disease onset, 135 and 48 patients had auricular involvement (59% of 229 patients, defined as auricular-onset subgroup; AO) and respiratory involvement (21%, respiratory-onset subgroup; RO), respectively. 46 patients presented with other conditions (20%, miscellaneous-onset subgroup; MO) including CNS, ocular, and inner ear involvement, among others.RO patients showed worse (poorer) prognostic stages than AO patients. MO patients developed respiratory and/or auricular involvement thereafter and then showed significantly higher mortality rate (15%; 7/46) than AO patients (5.9%; 8/135).In RP patients who did not develop respiratory involvement until the last follow-up (throughout the disease course; 117 patients), mortality rate was 19% in 26 MO patients and 3.3% in 91 AO patients. Accordingly, RO patients and MO patients associated with relatively poor prognosis compared with AO patients.
Conclusions:
Allocation of patients to RO and MO subgroups was suggested to associate with poorer prognosis of RP than AO subgroups, especially AO subgroups without respiratory involvement throughout. All RP patients deserve careful monitoring but special attention should be paid to MO patients because of their diverse and accelerated disease progression.
... Our patient had her first RP attack in 2013 with edema and swelling on the auricle. Edema develops in the airway during the active period of the disease, and fibrous tissue develops due to chronic inflammation in the late period and the airway collapses due to the destruction of the laryngotracheal cartilages [5]. The estimated incidence is 3.5/1,000,000/year [6]. ...
... Relapsing polychondritis is a systemic disease causing inflammation of cartilaginous structures in multiple organs [1]. Clinical phenotypes of relapsing polychondritis follow two patterns: either nasal cartilage/airway involvement or external ear involvement. ...
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].
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.
Background
Relapsing polychondritis (RP) is a rare autoimmune disease affected various cartilage, Patients with tracheal cartilage involvement are different from other patients. The objectives of this study were to allocated RP patients into two subgroups by chest computed tomography (CT) and compare the clinical features and disease patterns of each group.
Methods
A retrospective cohort study collected RP patients hospitalized at the Beijing Chao-Yang Hospital between January 2012 - August 2021. Patients were divided into two groups: respiratory involvement group and non-respiratory involvement group according to chest CT.
Results
In our study, respiratory involvement found in 59.7% (n=43) patients, which had higher rate of costochondritis, fewer rate of Inflammatory eye disease and auricular chondritis than those in non-respiratory involvement. Compared with non-respiratory involvement subgroup, The incidence of pulmonary infection marginally increased and those inflammatory indexes except for CAR were significantly higher in respiratory involvement subgroup, further subgroup analysis found that there was no significant relationship between inflammatory indexes and pulmonary infection. Finally, 5 patients died during the follow-up in this cohort with a median follow-up time of 6 years (range 3-8 years).
Conclusion
59.7% of patients had respiratory involvement according to chest CT findings in our cohort, which had a strong inverse relationship between respiratory and auricular, ocular involvement. Increase inflammatory indexes were not correlated with pulmonary infection, suggesting that patients with respiratory involvement had a higher disease activity index of RP. The probability of survival was not found significant in two subgroups.
Relapsing polychondritis (RPC) is a rare systemic immune-mediated disease characterized by recurrent inflammation of cartilaginous and proteoglycan-rich tissues throughout the body. Auricular, nasal, tracheal, and articular chondritis and arthritis are common systemic symptoms in patients with RPC. Ocular tissues are also targets of inflammation in RPC, and a variety of ocular symptoms are observed in approximately half of the patients with RPC. Scleritis/episcleritis, uveitis, and conjunctivitis are common symptoms associated with RPC. Less frequently, keratitis, retinopathy, optic neuropathy, muscle palsy, and orbital inflammation are also observed. Ocular inflammation could also be the first manifestation of RPC. Although RPC is a potentially fatal and sight-threatening disease, the rarity of the disease and its protean clinical presentation may lead to delayed diagnosis or misdiagnosis. Given the high prevalence of ocular involvement in RPC, to avoid misdiagnosis, physicians should be suspicious of RPC when they see patients with recurrent ocular inflammatory conditions and various systemic symptoms. In this article, we provide a comprehensive review of ocular manifestations associated with RPC.
Objective
The novel inflammatory markers C-reactive protein to albumin ratio (CAR), neutrophil to lymphocyte ratio (NLR), and platelet to lymphocyte ratio (PLR) were associated with the disease activity of many autoimmune diseases. The aim of this study was to evaluate the association of these new inflammatory indexes with relapsing polychondritis disease activity index (RPDAI).Methods
The data of relapsing polychondritis (RP) patients hospitalized between 2004 and 2020 at Peking Union Medical College Hospital were collected. One of the exclusive criteria was that RP patients overlapped with other diseases. Another was the RP patients with incomplete data. A total of 170 RP patients and 170 healthy controls (HCs) were included. The association of new inflammatory makers with RPDAI was assessed by Spearman’s correlation analysis.ResultsCompared to HCs, the CAR, NLR, and PLR were significantly higher in RP patients (both p < 0.001). The CAR, NLR, PLR, erythrocyte sedimentation rate and neutrophil counts in peripheral blood positively correlated with RPDAI. Blood albumin, lymphocyte count, hemoglobin (Hb) negatively correlated with RPDAI. The association of CAR, NLR, and PLR with RPDAI was demonstrated by Spearman’s correlation analysis.Conclusion
The novel inflammatory markers CAR, NLR and PLR were associated with RPDAI.
Key Points
• This is the first research to explore the association of CAR, NLR, and PLR with disease activity in patients with RP
• CAR, NLR, and PLR are positively correlated with RPDAI
• CAR, NLR, and PLR might be the potential predictors of disease activity in RP
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.
We read with great interest the very thoughtful letter from Dr. Zhang and colleagues and thank the authors for their interest in our study entitled “Defining Clinical Subgroups in Relapsing Polychondritis: A Prospective Observational Study”.
We read with great interest the article by professor Ferrada and colleagues (1). In this prospective observational cohort, the authors defined three subgroups of relapsing polychondritis (RP).
Background
Relapsing polychondritis(RPC) is a rare autoimmune disease, of which the diagnosis in early stage is challenging. Defining the clinical patterns and disease evolution may help early recognition of this rare disease.
Results
Sixty-six males and 60 females were included with onset age of 47.1±13.8 years and followed up for a median of 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 subdivided as limited and systemic form of RPC (27.8% with limited form of Ear pattern and 24.6% with limited form of Airway pattern). During follow-up, a small part of patients with Ear pattern and Airway pattern progressed into Overlap pattern and some Airway-Ear negative pattern patients progressed into Ear pattern. Most of the limited RPC patients remained limited form during follow-up while some patients with limited form progressed into systemic form. Patients with Ear pattern had the highest survival rate and relative 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, and clinical patterns and presenting forms can evolve during follow-up. Our findings may facilitate early recognition of this rare disease and contribute to an updated classification criteria covering all the clinical spectrum of RPC
Objective
To assess patient-reported burden of disease in pediatric patients with relapsing polychondritis (RP) and to compare these findings to adult patients.
Methods
A survey based on known clinical symptoms of RP was developed and administered to patients with a pediatric diagnosis of RP. Adult patients completed a similar survey.
Results
Twenty-one pediatric patients, or their parents completed surveys. Median age at symptom onset was 6 (IQR=1.8-12). Prior to diagnosis, most pediatric patients went to the emergency room (61.9%), saw >3 physicians (57.1%), and took >1 year to be diagnosed (61.9%). Pediatric patients were often diagnosed with asthma (42.9%), ear infections (42.9%), or sinusitis (33.3%) prior to diagnosis of RP. Symptoms prior to diagnosis included ear pain/redness (85.7%), joint pain/swelling (61.9%) and airway symptoms (38.1%). Four pediatric patients (19%) reported tracheomalacia requiring tracheostomy. Pediatric patients frequently missed school due to their disease (71.4%). Surveys from 290 adult patients were compared to pediatric patients. Pediatric patients were significantly more likely to undergo biopsy (42.9% vs 17.4%; p<0.01) and be treated with biologics (42.9% vs 19%; p=0.02). Adults were significantly more likely to be female (87.8% vs 28.6%; p<0.01) and to report airway symptoms (77.9% vs 47.6%; p=0.01). Prevalence of disease complications was not significantly different between adult and pediatric patients.
Conclusion
The burden of disease in pediatric patients with RP includes missed school, diagnostic delay, emergency room visits, and multi-system disease, with resultant damage to cartilaginous structures. Differences in airway involvement and treatment approaches may exist between pediatric and adult patients.
The aim of this paper is to present a case of relapsing polychondritis and response to cyclophosphamide pulse therapy. A 19 years old female patient presented with inflammatory back pain, asymmetrical oligoartheritis and acute anterior uveitis. CT thorax revealed edema in the glottis and trachea causing narrowing of lumen. Based on the investigations and clinical finding a final diagnosis of relapsing polychondritis was done and started with 5 pulse of cyclophosphamide which showed improvement. Relapsing polychondritis is a rare condition commonly affecting 40-50 years age group. But can be seen in younger age too in both the sexes. Laryngobronchial involvement occurs in approximately 50% of patients, and is among the most serious compli- cations. Here we present the case of a 19 year old female with relapsing polychondritis presenting with stridor due to tracheal involvement.
Objective:
Recently we developed the Rasch Everyday Activity Limitations (REAL) generic item bank for measuring physical function. In this study we evaluate the REAL item bank in 209 RA patients and demonstrate how computerized adaptive testing (CAT) and Optimal Test Assembly methods can be used to derive measures from the REAL item bank with superior measurement performance compared with the HAQ Disability Index (HAQ-DI).
Methods:
Structural validity of the item bank was assessed using confirmatory factor analysis. The validity of the REAL score metric in RA was evaluated by examining differential item functioning against the general population calibration sample. Besides the REAL-CAT, a 6-item short form (REAL-6) was developed using Optimal Test Assembly that was optimized with respect to common disability levels in RA, content and reading ease. Measurement precision of the different instruments was examined using item response theory methods. Construct validity was evaluated by testing hypothesized correlations with external measures.
Results:
Good model-data fit was observed for a one-dimensional model and only two items showed differential item functioning of substantial magnitude. The REAL-CAT had superior measurement precision compared with HAQ-DI and REAL-6. REAL-6 outperformed HAQ-DI across all but the very lowest level of physical function. All three instruments demonstrated good construct validity (>75% of hypotheses affirmed) and only HAQ-DI had a ceiling effect (23.9%).
Conclusion:
This study supports the validity of the REAL item bank and illustrates the potential of CAT and OTA applications based on the REAL item bank for assessing physical function in RA.
In this issue of Arthritis Care & Research, the authors of Patient‐Perception of Disease‐Related Symptoms and Complications in Relapsing Polychondritis report for the first time the patient's view over the disease (1). Thanks go to them for this important step forward. The method used however, carries some limitations, as indicated very honestly by the authors.
This article is protected by copyright. All rights reserved.
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.
This work is aimed to study the clinical and prognostic features of relapsing polychondritis (RP) in China. A total of 158 RP cases from 1985 to 2013 in China were included and compared with international case series in terms of clinical features, systemic involvement, differential diagnosis and prognosis. (1) The average age at the onset was 45.3 years old, the average age for initial symptoms was 14.4 months, female/male ratio was 0.7:1 and misdiagnosis rate was 47 %. (2) The incidence of arthritis was lower than that in Caucasians. The incidences of auricular chondritis (68 %: 84-95 %), ocular inflammation (44 %: 49-65 %) and renal involvement (3 %: 7-26 %) were lower, and laryngotracheal symptoms (69 %: 31-67 %), skin (46 %: 4-38 %) and neurological involvement (12 %: 2-8 %) were higher during the follow-up period. The proportion of associated autoimmune disease and systemic vasculitis were 5 and 3 %, respectively, similar to that in Japanese (4 and 2 %), but less than that in Caucasians (12-31 and 8-18 %) except the Francès's study (7 and 3 %). The primary death cause is respiratory failure due to RP, followed by lung infections and cardiovascular events. (3) Juvenile RP (onset ≤18 years) was more severe than adults, similar to results from the Caucasians. However, Chinese juvenile RP had more severe ocular inflammation (57 %: 40-47 %), arthritis (100 %: 71-90 %), cardiovascular (14 %: 3-10 %) and skin involvement (20 %: 10-11 %) than Caucasian juvenile RP. Although sharing most of the clinical features with case series in previous literature, Chinese patients with RP have its unique characteristics.
Relapsing polychondritis (RP) is a rare autoimmune disease affecting the multiple organ system. Here, we describe a case of RP initially presenting with high fever. The patient was referred to our hospital for further examination of fever of unknown origin (FUO). On admission, the patient reported dry cough in addition to fever. On physical examination, her red, swollen ears were noted, attributed on histology to inflammation with auricular perichondritis. She was diagnosed with RP and treated with oral prednisone (50 mg/day); her fever and auricular inflammation resolved. The patient no longer reported cough and body temperature returned to normal and the elevated levels of C-reactive protein (CRP) were normalized. In this case, identification of the origin of fever was a challenge because of unspecific symptoms; however, awareness of the systemic manifestations of RP may lead to the prompt diagnosis and therapeutic intervention.
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.
Background: Relapsing polychondritis is a rare disease characterised by inflammation of cartilaginous and proteoglycan rich structures. As there are only a few published single centre case series from all across the world, we describe our experience with 26 patients at a tertiary centre in north India.
Methods: A retrospective study with all patients meeting Damiani and Levine's modification of McAdam's diagnostic criteria. Clinical details, investigations, disease activity assessment (Relapsing Polychondritis Disease Activity Index(RPDAI), treatment and outcomes were recorded.
Results: Ten men and sixteen women (median age 45 years) met the diagnostic criteria. Auricular chondritis (96%), arthritis (54%), hearing impairment (42%), ocular(42%), dermal (26%), cardiovascular(11%) and laryngotracheal involvement(11%)characterized the clinical presentations. The median RPDAI was 31(range 9-66). Two patients died during observation. Overall survival was 92.3% (median survival 13.5 years).
Conclusions: Apart from reduced laryngotracheal involvement, RP in India was clinically similar to recorded patterns elsewhere.
A 31-year-old woman was referred to chest clinic with an 18-month history of cough, breathlessness and weight loss. She had previously been treated unsuccessfully with bronchodilators. In clinic she was found to have vocal hoarseness and harsh, monophonic inspiratory breath sounds. A bronchoscopy was arranged, but the patient was found dead before it could be performed. A postmortem examination with histology found evidence of active and chronic inflammation as well as destruction of cartilage, in keeping with a diagnosis of relapsing tracheobronchial polychonditis.
Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation and destruction of the cartilaginous structures. Tracheobronchial chondritis is a dreaded complication of RP. We wish to report a case of RP of the trachea and bronchi which was treated with nasal continuous positive airway pressure.
A 29-year-old female with a history of relapsing polychondritis (RP) and open repair of a proximal descending thoracic aneurysm presented with 2 areas of asymptomatic thoracic aortic aneurysmal dilatation. The patient returned 3 months later with symptomatic aneurysm expansion, and she underwent ascending aortic arch replacement. She subsequently underwent staged endovascular repair of the distal descending thoracic aorta. RP is a rare disorder with an incidence of 3.5 per million persons annually, 4% to 7% of whom develop aneurysmal disease. Because of the aneurysmal potential of this disease, it is important for vascular surgeons to be aware of its presentation and treatment. To our knowledge, this is the first reported case describing endovascular technique to treat such a patient.
A case with relapsing polychondritis is described where primary involvement of the aortic valve cusps produced severe aortic regurgitation requiring valve replacement. An aneurysmal dilatation of the ascending aorta developing later led to disruption of the prosthesis requiring re-operation. Superior vena caval obstruction, an abdominal aortic aneurysm which ruptured and required resection, and obstructive lesions in common iliac arteries, presumably the result of the same process that involved the aorta and the cartilaginous structures, were also seen.
Relapsing polychondritis is rare and its cause is unknown. The tissues affected are those with a high glycosaminoglycan content, such as cartilage, the aorta, the sclera and cornea, and parts of the ear. Symptoms can usually be controlled with oral steroids, but when there is coexistent progressive crescentic glomerulonephritis quadruple chemotherapy may be used. Three cases of the clinical syndrome of relapsing polychondritis were studied in which rapidly progressive cresentic glomerulonephritis developed. In two the patients appeared to respond to aggressive treatment with immunosuppressive agents and anticoagulants. The multisystemic nature of the disease, the renal lesions, and the response to treatment all suggested that the condition might be related to periarteritis nodosa.
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.
Relapsing polychondritis is a rare multisystemic connective tissue disorder found mainly in Caucasians. Oriental patients with relapsing polychondritis are uncommon. We report 2 Chinese patients with relapsing polychondritis who had severe laryngotracheal narrowing requiring tracheostomy. Unlike most patients, there was marked costal chondritis resulting in depression of the anterior chest wall with minimal involvement of other joints. One of the patients had treatment with prednisolone, dapsone, azathioprine, cyclophosphamide and cyclosporin A at various stages of the disease without significant remission of the disease.
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.
Relapsing polychondritis is a disease of unknown cause in which both articular and nonarticular cartilages represent the sites of primary pathologic change. We have reported the 44th new case—a case in which affection of the articular cartilages dominated an extremely severe clinical course. The multiple variation in the clinical appearance and the grave prognosis have earned for this disease an enthusiastic interest among a great variety of clinical specialists.
The reported incidence of mitral regurgitation in relapsing polychondritis ranges from 2 to 3% and is associated with aortic regurgitation. There are no reports that mitral regurgitation can be an isolated cardiac complication of relapsing polychondritis. This case report demonstrates that partial chordal rupture and consequent severe mitral regurgitation can be the only features of cardiac involvement in relapsing polychondritis.
Relapsing polychondritis (RP) is a rare disease characterized by recurrent inflammation and destruction of the cartilaginous structures. Tracheobronchial chondritis is a dreaded complication of RP. We wish to report a case of RP of the trachea and bronchi which was treated with nasal continuous positive airway pressure.
The purpose of this study was to report a case of bilateral blindness, bilateral necrotizing scleritis, and bilateral deafness in a patient with myelodysplastic syndrome (MDS). In such a patient, the possibility of relapsing polychondritis (RPC) associated with MDS must be considered. CASE REPORT/METHODS: A 66-year-old patient suffered from myelodysplastic syndrome (MDS). Shortly before his death, he became bilaterally blind and deaf. A biopsy was taken from the conjunctiva and the bone marrow, and both eyes were obtained after death for further investigation. Findings of the clinical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented.
Ocular sections showed a diffuse necrotizing scleritis with moderate uveitis and no identifiable infectious agent. Neither was there any evidence of a leukemic infiltration. The deafness had been due to inner ear failure, and the patient died of a cardiac failure.
Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC). Furthermore, RPC can be associated with MDS. Thus, in addition to leukemic infiltration and infection involving ocular structures, ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential complications.