Chondritis

Abstract: 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.

Abstract: 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.

Abstract: Relapsing polychondritis is a disorder of unknown cause characterized by the destruction of cartilage. To test the hypothesis that immunologic mechanisms are involved in the pathogenesis of relapsing polychondritis, we analyzed the serum of 15 patients for the presence of antibodies to cartilage. Antibodies to Type II (cartilage) collagen were found in the serum of five patients at the time of acute symptoms. No antibodies were detected either to cartilage proteoglycan or to other collagen types. The antibodies were detected at the onset of the disease and their titers appeared to correlate with severity of disease. Circulating immune complexes were also detected in the serum of these patients. Our findings support an immunologic involvement in this condition.

Abstract: Objective. In this study we describe clinical and immunogenetic findings in 62 unselected patients with relapsing polychondritis. Methods. 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. Results. 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). Conclusion. 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.