Jaccoud's arthritis.

Abstract

In 1869 Jaccoud described an unusual case of deforming arthritis appearing in a patient following frequent severe attacks of rheumatic fever. The deformities were primarily in the hands and characterized by muscular atrophy with severe ulnar deviation and flexion at the metacarpal-phalangeal joints. Bywaters in 1950 reported a similar case and reviewed the European literature which suggested that this entity is distinguishable from rheumatoid arthritis (1). As this condition, to our knowledge, has not appeared in the radiological literature, it is the purpose of this paper to present the radiological aspects observed in 2 patients studied within the past year at Georgetown University Hospital in whom clinical changes were demonstrated, similar to those described by Jaccoud. It is hoped that this report will enable radiologists to recognize the condition and suggest the diagnosis. Case Reports Case I: A 26-year-old, white male with exertional dyspnea was admitted to Georgetown University Hospital for cardiac evaluation. At the age of fourteen he had been hospitalized because of migratory joint pain and swelling associated with fever. On treatment with ACTH and physical therapy these acute joint symptoms were alleviated and did not return. During the next twelve years the patient noted a slowly progressive ulnar deviation and flexion deformity of the fourth and fifth metacarpal-phalangeal joints. These changes were most marked in the right hand. From 1958 to 1961 there were three subsequent hospital admissions for fatigue and increasing dyspnea. During his last admission the patient was evaluated for cardiac surgery because of significant mitral and aortic valvular involvement, but he was considered a poor risk for any surgical procedures. On the most recent admission the patient presented as a thin, white male appearing chronically ill. The marked deformities of the hands consisted of fusiform swelling of the metacarpal-phalangeal joints with flexion deformity and ulnar deviation (Fig. 1). The palmar fascia was not involved. There was also a hyperextension deformity of the proximal interphalangeal joints. The ulnar deviation could be easily corrected voluntarily by slipping the tendons laterally over the metacarpal-phalangeal joints. Roentgenograms demonstrated a soft-tissue swelling about the metacarpal-phalangeal and interphalangeal joints and there was narrowing of all the metacarpal-phalangeal joints with subluxation of the fifth on the right (Fig. 2). A biopsy of the synovial and articular capsule of the involved metacarpal-phalangeal joints showed no evidence of rheumatoid synovitis. There was, however, periarticular fibrosis without synovial involvement. Significant laboratory findings showed the test for rheumatoid factor (bentonite flocculation) and lupus erythematosus cell preparations to be negative. Repeated antistreptolysin titers were normal and the VDRL test was negative.