Jaccoud’s Arthropathy
A 58-year-old man presented with a 15-year history of systemic lupus erythematosus (SLE) that was characterized by polyarthritis, photosensitivity, malar rash, and pleuritis. Laboratory workup revealed antinuclear antibodies, anti–double-stranded DNA antibodies, anti-Ro (SS-A) antibodies, non–nephrotic-range proteinuria, and leukopenia. Testing for rheumatoid factor and anti-CCP antibodies was negative. Despite treatment with glucocorticoids, hydroxychloroquine, and azathioprine, hand deformities, including swan neck deformity, Z deformity of the thumb, and ulnar deviation of the fifth digit, developed over a 10-year period (Panel A and video). These deformities were reducible and are characteristic of Jaccoud’s arthropathy. Magnetic resonance imaging of the hands showed no erosions (Panel B). Although classically described in rheumatic fever, Jaccoud’s arthropathy has also been described in SLE and other diffuse connective-tissue diseases. In Jaccoud’s arthropathy, the deformities result mainly from soft-tissue abnormalities, such as laxity of ligaments, fibrosis of the capsule, and muscular imbalance, rather than from destruction of the bone of joints, as occurs in rheumatoid arthritis. The mainstay of the management of Jaccoud’s arthropathy includes physical therapy and the use of orthotic devices. Because this patient is a taxi driver, the hand deformities have been an important limitation to his capacity for work, despite physical therapy for muscle strengthening. Soft-tissue surgery for the correction of the hand deformities is under consideration but has not been performed.