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CFP Relapsing Polychondritis
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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 (RP) is a rare multisystem autoimmune disease of unknown origin characterized by recurrent episodes of inflammation and progressive destruction of cartilaginous tissues. Elastic cartilage of the ears and nose, hyaline cartilage of peripheral joints, vertebral fibrocartilage and tracheobronchial cartilage, as well as proteoglycan-rich structures of the eye, heart, blood vessels or inner ear may all be affected. In most patients RP manifests in a fluctuating but progressive course which eventually results in a significant shortening of life expectancy. The relatively uncommon occurrence, the unknown etiopathogenesis, the ambiguous clinical pattern, as well as the variety in its course and response to therapy may all contribute to the difficulties the physician must overcome when managing RP. Beside describing the main features of RP and seven clinical cases of our own, in the present review we focus on recent findings in the etiopathogenesis and novel treatment options.
The objective of this study was to characterize the clinical features of relapsing polychondritis (RPC) within the Department of Defense beneficiary population and determine the utility of echocardiography, imaging studies, and pulmonary function testing for diagnosis and monitoring disease.
We performed a retrospective Electronic Medical Record chart review of all patients diagnosed with RPC within the Department of Defense between January 2004 and December 2009.
Thirty patients met McAdam's diagnostic criteria and an additional 13 met our criteria for partial RPC. Auricular chondritis (88%), inflammatory eye disease (57%), and arthritis (60%) were the most common clinical manifestations. Pulmonary involvement was seen in 16 (37%) patients. Methotrexate (42%) and corticosteroids (21%) were the most conventional therapies. Thirty (70%) patients had pulmonary function tests with flow volume loop abnormalities observed in 33%. Chest computed tomography was performed in 63%, with abnormalities in 48%. Abnormalities on echocardiography were observed in 12 of 25 (48%) patients.
The incidence, demographic data, and organ involvement in our RPC patients were similar to previous studies. The diagnosis of RPC was determined primarily on physical examination and symptom-driven diagnostic testing. There was no notable pattern by rheumatologists for monitoring the progression of tracheobronchial tree or large vessel involvement. Interpreting flow volume loops is recommended with pulmonary function testing to detect early laryngotracheal involvement. Computed tomography of the chest is also recommended to monitor for vascular and tracheobronchial tree involvement.
This article is aimed to review of relapsing polychondritis (RP) and its association to hepatitis C virus (HCV) infection. A case of RP associated with HCV infection in a 59-year-old male is reported. The English medical literature was reviewed for RP and its association with HCV infection. RP is a rare autoimmune and multisystem disorder of unknown etiology in which the cartilaginous and related tissues are the primary targets of inflammation. HCV infection is a more common systemic illness with known hepatic and extra-hepatic manifestations. Although RP is associated with other diseases in about 35% of cases, only one case of RP, HCV, and mixed cryoglobulinemia has been reported. We report a case of RP associated with HCV infection. Treatment with pegylated interferon and ribavirin resulted in sustained virologic response and remission of treatment-resistant RP with azathioprine. We report a case of RP and associated HCV infection. Although treatment of HCV infection resulted in remission of RP, it is unknown if there is a causal relationship between HCV infection and RP.
Relapsing polychondritis (RPC) is a rare autoimmune disease in which the cartilaginous tissues are the target for inflammation and destruction, the associated immune reaction causes inflammation in non-cartilaginous tissues like kidney and blood vessels. This article provides a description of a case of RPC and a review article about the disease.
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.
Relapsing polychondritis is a rare disease of unknown etiology. There are approximately 211 reported cases in the world literature. This is a report of ten cases from the Cleveland Clinic Foundation.
McAdam's diagnostic criteria for R.P. were reviewed and modified. For diagnosis, all patients had to have 1. at least three or more diagnostic criteria, histologic confirmation not necessary; 2. one or more of McAdam's signs with positive histologic confirmation; or 3. chondritis in two or more separate anatomic locations with response to steroids and/or Dapsone.
Chondritis of the auricles (9/10 patients) and arthropathy (8/10 patients) are the most common presenting signs. Chondritis was also seen in the nose (6/10) and the upper respiratory tract involving the larynx and trachea (4/10). Cochlear and vestibular damage and ocular inflammation were each seen in 5/10 patients.
Patients were treated with steroids and/or Dapsone. Both drugs were reliable in abating episodes of activity and in decreasing recurrences. These results further support Dapsone as an alternate form of treatment for RP.
Relapsing polychondritis, an uncommon, chronic, multisystem disorder characterized by recurrent episodes of inflammation of cartilaginous tissues, can be life-threatening, debilitating, and difficult to diagnose. This review is based on the authors' experience with 36 patients with relapsing polychondritis who were followed from 1980 to 1997, 30 patients located elsewhere who completed a detailed questionnaire and interview, and a perusal of English-language textbooks and papers located by a systematic search of the MEDLINE database. Relapsing polychondritis can present in a highly ambiguous fashion; therefore, in the authors' series, the mean delay from the time medical attention was sought because of symptom onset until diagnosis was 2.9 years. Although prednisone was the main form of treatment, methotrexate seemed to be of additional value. Survival was much more favorable than previously thought. Greater awareness of relapsing polychondritis would probably lead to earlier diagnosis and better outcomes.
A red eye is the most common ocular disorder that primary care physicians encounter. Most cases are relatively benign. Some, however, herald a vision-threatening or even life-threatening disorder. A detailed description of the full differential diagnosis of a red eye is available elsewhere.1 This overview does not assume that the examiner has access to a slit lamp or has been trained to use it. Subconjunctival Hemorrhage A subconjunctival hemorrhage (Figure 1) is often the cause of acute ocular redness. The diagnosis is based on simple observation of the characteristic features of such a hemorrhage: the redness, which is unilateral, is . . .
Relapsing polychondritis is a unique, rare autoimmune disorder in which the cartilaginous tissues are the primary targets of destruction but the immune damage can spread to involve noncartilaginous tissues like the kidney, blood vessels, and so forth. The manifestations of the disease can take many different forms and the pathogenesis is still unclear. It may occur in a primary form or it may be associated with other disease states. This article summarizes important aspects of the disease with a focus on recent information regarding clinical manifestations, disease associations, pathogenesis, and advances in therapeutics.
For cellulitis that does not respond to conventional antimicrobial treatment, clinicians should consider, among other explanations, several noninfectious disorders that might masquerade as infectious cellulitis. Diseases that commonly masquerade as this condition include thrombophlebitis, contact dermatitis, insect stings, drug reactions, eosinophilic cellulitis (the Wells syndrome), gouty arthritis, carcinoma erysipelatoides, familial Mediterranean fever, and foreign-body reactions. Diseases that uncommonly masquerade as infectious cellulitis include urticaria, lymphedema, lupus erythematosus, sarcoidosis, lymphoma, leukemia, Paget disease, and panniculitis. Clinicians should do an initial diagnostic work-up directed by the findings from a detailed history and complete physical examination. In many cases, skin biopsy is the only tool that helps identify the correct diagnosis. Special tests may also be needed.
Relapsing polychondritis is a rare disease most commonly presenting as inflammation of the cartilage of the ears and nose. Auricular chondritis, with red ears resembling infectious cellulitis, is the most common initial finding. Antibodies to type II collagen in cartilage are found, and the earlobes are classically spared. Chronic disease may result in a flabby, droopy ear, cauliflower ear, or saddle nose deformity. Acute involvement of the tracheal cartilage may cause collapse of the airway with obstruction and pulmonary infections. Arthritis may be oligoarticular or polyarticular, most often involving the costochondral junctions. Other manifestations include audiovestibular damage; heart valve disease; and neurologic, ocular, and renal disease. Corticosteroids remain the major treatment. Other therapies include nonsteroidal anti-inflammatory drugs, dapsone, colchicine, azathioprine, methotrexate, cyclophosphamide, hydroxychloroquine, cyclosporine, and infliximab.
A previously healthy 59-year-old man was admitted to this hospital with fever, weight loss, and painful swelling of both eyes and the right ear. Six weeks earlier, headache and fever had developed, followed by pain in the neck, pain and swelling of the eyes, and pain in the right ear; symptoms did not respond to antibiotic therapy. Laboratory studies on admission disclosed an elevated erythrocyte sedimentation rate and anemia. A diagnostic procedure was performed.
- X Puéchal
- B Terrier
- L Mouthon
- N Costedoat-Chalumeau
- L Guillevin
- Le Jeunne
- C Relapsing
Puéchal X, Terrier B, Mouthon L, Costedoat-Chalumeau N, Guillevin L, Le Jeunne C.
Relapsing polychondritis. Joint Bone Spine 2014;81(2):118-24. Epub 2014 Feb 18.
Cet article a fait l'objet d'une révision par des pairs
This article has been peer reviewed.
Cet article a fait l'objet d'une révision par des pairs.
Can Fam Physician 2018;64:363-7