Jaccoud's Arthropathy with Radial Shaft Deformity in a Patient with a History of Mild Carditis and Sydenham's Chorea

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Background: Jaccoud’s arthropathy was first described in 1869 in a patient with recurrent attacks of acute rheumatic fever and recurrent migratory polyarthritis. The typical features include characteristic joint deviation with metacarpophalangeal subluxation, ulnar deviation, swan neck deformity of the fingers, and Z deformity of both thumbs and hook formation and alignment disorders without noticeable erosions on radiographs. In this report, Jaccoud’s arthropathy presenting with deformity of shaft of the radius is described. Case presentation: A 12-year-old-girl was diagnosed to have chorea at the age of 8 years after she presented with irregular, jerky, rapid and involuntary movements that involved the face, the trunk and the distal extremities. She was treated with haloperidol. The chorea resolved after 3 months of treatment. Echocardiogram showed thickening of the mitral valve leaflets and mild mitral regurgitation. Three years later, the child presented with progressive symmetric deformity of the wrist joints. She reported no pain or limitation of movement. On physical examination, there was no cardiac murmur. There was varus deformity of both wrist joints. There was no tenderness or limitation of movement. Anti-streptolysin O was negative at the first presentation and on this visit. Rheumatoid factor was negative but antinuclear antibody test was positive. Echocardiogram showed complete resolution of the mitral regurgitation. X-ray of the wrists showed marked symmetric curving of the distal radius and varus deformity of the wrists. There was joint deviation with metacarpophalangeal subluxation and ulnar deviation of the hand. There was no Z deformity of the thumbs. Conclusion: The common presentation of Jaccould’s arthropathy is involvement of the wrist, metacarpophalangeal joints and joints of the feet. The metacarpal and metatarsal bones are the ones, which are commonly affected. Our patient has an unusual presentation of deformity of the distal radial bone. This has not been described, at least to our knowledge.