RELAPSING POLYCHONDRITIS - CLINICAL AND IMMUNOGENETIC ANALYSIS OF 62 PATIENTS

Objective. in this study we describe clinical and immunogenetic findin gs in 62 unselected patients with relapsing polychondritis. Methods. I n a multicenter study, clinical data of 26 (41.9%) female and 35 (58.1 %) male patients were collected. HLA-DR specificities were identified in 60, and the frequencies were compared with those in healthy control s. Results, The median age at the rime of diagnosis was 46.6 years (ra nge 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 sys tem (22.6%), central nervous system (9.7%), and kidneys (6.5%) was fou nd as well. 22 (35.5%) patients had associated diseases such as system ic lupus erythematosus or rheumatoid arthritis. Susceptibility to rela psing 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 ). Conclusion. Immunogenetic findings as well as similarities and over lapping clinical symptoms with other autoimmune or rheumatic diseases suggest that immunological mechanisms play a major role in the pathoge nesis of relapsing polychondritis.