RARE RHEUMATIC DISEASES. RELAPSING POLYCHONDRITIS: A CLINICAL CASE

Abstract

The diagnosis of a disease accompanied by febrile syndrome presents special problems in clinical practice. The traditional diagnostic algorithm in this case involves the exclusion of infections, cancer, and rheumatic diseases. The causes of fever can be severe types of rheumatoid arthritis, systemic vasculitis, and diffuse connective tissue diseases, as well as rare diseases, the difficulty of diagnosis of which is due to low awareness of the disease and the absence of specific clinical, laboratory, and instrumental signs. These also include relapsing polychondritis (RPC) in particular. RPC is a rare systemic inflammatory disease of cartilage tissue, which leads to its structural changes resulting in complete destruction. Cartilages of the acoustic apparatus, nose, trachea, and joints are mainly affected. The severity of cardiovascular injury in RPC has recently been emphasized. This disease is observed with equal frequency in men and women, primarily at the age of 40–60 years, predominantly in white people, but it also occurs in other populations. The etiology of RPC is unknown. There are no pathognomonic laboratory and instrumental tests for the diagnosis of RPC. A unified approach to treating this condition has not been elaborated. The available data on the pathogenesis, various clinical manifestations and treatment options of the disease are analyzed. The authors’ own observations of RPC are depicted.