Jaccoud's syndrome. A nosographic entity associated with systemic lupus erythematosus

Six Scandinavian cases of Jaccoud's syndrome, resulting from systemic lupus erythematosus (SLE) are described; 1 patient suffered from both SLE and rheumatoid arthritis (RA). Clinically, the patients showed hand deformities similar to those of RA with pronounced ulnar deviation and swan-neck configuration. Clinically, however, the hands in cases of Jaccoud's syndrome differ from those in RA because the deformities develop more slowly, without much pain, and hand function is well-preserved. Radiologically, subluxation develops in the metacarpophalangeal joints without the typical rheumatoid joint-associated osseous destruction. The presence of erosions and hooks are exceptional and, if present, they are not typical of RA. It is important that Jaccoud's syndrome be recognized and differentiated from RA because an incorrect diagnosis could lead to a choice of therapy inappropriate for patients with SLE.