Przewlekła poreumatyczna artropatia — zespół Jaccouda u 21-letniego chorego

Abstract

Jaccoud’s arthropathy (JA) is a condition characterised clinically by ‘reversible’ joint deformities resulting from soft tissue abnormalities, rather than the destruction of bone joints. Although rare, it has been described in patients with chronic rheumatic heart disease. It typically involves metacarpophalangeal and metatarsophalangeal joints. JA has also been described in systemic lupus erythematosus (SLE), infections and neoplasia. Here, we report the case of a 21 year-old male who had presented with a 16-year history of polyarthralgia, deformities of the hands for the last four years, and a three-year history of exertional palpitation and dyspnoea. Physical examination revealed peripheral signs of aortic run off, with hyperdynamic left ventricle and chronic severe aortic and mitral regurgitation. On examination, gross deformities were noted in both hands in the form of ulnar deviation, swan neck, and ‘z’-thumb, involving interphalangeal joints, metacarpophalangeal and first carpometacarpal joints which were partially correctable. Laboratory investigations ruled out rheumatoid arthritis, SLE, and acute rheumatic fever. Chest X-ray, electrocardiogram and two-dimensional echocardiography and Doppler study were consistent with chronic severe aortic regurgitation and severe mitral regurgitation. Radiographs of both hands showed posterior subluxation of the carpometacarpal joints, which were completely correctable. JA involving a carpometacarpal joint is exceedingly rare.