Background/Purpose: Relapsing polychondritis (RP) is a rare condition characterized by recurrent inflammation of cartilaginous tissues and systemic manifestations. Data on prognosis available from the latest large series published in 1986 indicate a 5- and 10-year survival after diagnosis of 74% and 55%, respectively [1]. The purpose of this study is to describe a recent series of patients with RP.

Methods: 142 patients with a diagnostic of RP according to Michet’s criteria were included in this monocentric retrospective study. All the patients were seen at least once since 2000. The median year of diagnosis was 2002 (1974 to 2011).

Results: 56 men (39%) and 86 female (61%) were included. The mean age at first symptoms was 43.5±15 years, and was higher for men (47±15 vs 41±15 p=0.02). The median delay of diagnosis was 1 year (1 to 25). The mean number of initial symptoms was 1.85±1, and main first symptoms consisted of nasal or auricular chondritis (59%), joint (32%), ophthalmologic (18%), general (15%) or cutaneous (15 %) manifestations. During a median evolution of 11 years (1 to 41), all the patients presented chondritis: auricular in 89%, nasal in 63%, laryngeal in 43%, chondrocostal in 40%, and tracheobronchial in 22%. Nasal and ear deformations occurred in 15 and 10% respectively. Other manifestations were articular in 69%, ophthalmological in 56% (episcleritis or scleritis in 41%, uveitis in 13%, keratitis in 4%), cochleovestibular in 34%, (deafness in 27% and vestibular syndrome in 20%), cardiac in 27% (including aortic valve insufficiency in 17% and cardiac surgery in 5%), cutaneous in 30% and neurological in 11%.

A concomitant hematological disease was present in 14%: myelodysplasic syndrome in 8% and others in 6%. Myelodysplasic syndrome affected only men in our series (p=8×10^-6), and was associated with older age (63 vs 41years; p=4×10^-6) and cutaneous involvement (p=0.0001). Deep vein thrombosis occurred in 11% of the patient.

58% of the patients had an inflammatory syndrome during disease’s flares with a median CRP level of 80 mg/l (10 to 379 mg/l). Antinuclear antibodies were found in 20%, anti-neutrophil cytoplasmic antibodies in 10%, rheumatoid factor in 13% (17/129).

An autoimmune disease was associated in 22% (including Sjogren syndrome in 9%, autoimmune thyroiditis in 6%, systemic lupus erythematosus in 4%).

Corticosteroids were used in 94%, methotrexate in 49%, dapsone in 38%, colchicine in 32%, azathioprine in 25%, mycophenolate mofetil in 19%, cyclophosphamide in 17%, TNF-alfa inhibitors in 11%, rituximab in 7%, anakinra in 3% and autologous stem cell transplantation in 2%.During the evolution, 18% were hospitalized for an infection and 13% were transferred in Intensive Care Unit.

To avoid selection bias, we studied the survival in the 87 patients who had a diagnosis of RP after 2000. After a median follow up of 7 years, the 5- and 10-years survival after diagnosis were of 95% and 91%, respectively. 

Conclusion: Compared with previous studies, the prognosis of RP has greatly improved.

[1]: Michet et al. Ann Intern Med. 1986;104:74-8.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/description-of-142-cases-of-relapsing-polychondritis-followed-in-a-single-center-since-2000/