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Relapsing polychondritis: clinical and immunogenetic analysis of 62 patients.
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1997
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The study aims to describe clinical and immunogenetic characteristics of 62 unselected relapsing polychondritis patients in a multicenter cohort. The authors performed HLA‑DR typing on 60 patients, compared allele frequencies with healthy controls, and collected clinical data including median age at diagnosis. Patients exhibited widespread organ involvement, most commonly auricular chondritis (93.5 %), ocular (50 %) and nasal (56.5 %) symptoms, with 35.5 % having associated autoimmune diseases; HLA‑DR4 was significantly linked to disease susceptibility, while HLA‑DR6 was inversely related to organ involvement, underscoring a key immunogenetic role.
In this study we describe clinical and immunogenetic findings in 62 unselected patients with relapsing polychondritis.In a multicenter study, clinical data of 26 (41.9%) female and 36 (58.1%) male patients were collected. HLA-DR specificities were identified in 60, and the frequencies were compared with those in healthy controls.The median age at the time of diagnosis was 46.6 years (range 17 to 86). 58 (93.5%) patients had auricular chondritis, 31 (50.0%) ocular symptoms, 35 (56.5%) nasal involvement. Involvement of joints (53.2%), respiratory system (30.6%), skin (24.2%), cardiovascular system (22.6%), central nervous system (9.7%), and kidneys (6.5%) was found as well. 22 (35.5%) patients had associated diseases such as systemic lupus erythematosus or rheumatoid arthritis. Susceptibility to relapsing polychondritis was significantly associated with HLA-DR4 (p < 0.001). There was no difference in the frequency or distribution of DRB1*04 subtype alleles between patients and healthy controls. The extent of organ involvement was negatively associated with HLA-DR6 (p < 0.011).Immunogenetic findings as well as similarities and overlapping clinical symptoms with other autoimmune or rheumatic diseases suggest that immunological mechanisms play a major role in the pathogenesis of relapsing polychondritis.