Case history

A boy aged 12 years presented to the local general hospital with a four week history of fever, rash, and arthralgia in September 1982. The rash was migratory and appeared urticarial, the most commonly affected sites were his trunk and thighs. The arthropathy was asymmetrical and mainly affected medium and large joints. At presentation he complained of discomfort in his ankles, left knee, and right wrist.

Clinical examination confirmed the presence of the rash and active synovitis of his right wrist. There was no evidence of lymphadenopathy, organomegaly or cardiac murmurs. Initial investigations included a raised erythrocyte sedimentation rate (ESR) at 34 mm 1st h and a moderate leucocytosis. Radiology of the affected joints was unremarkable and his electrocardiogram was normal. His rheumatoid factor and anti-nuclear antibodies were negative. An infection screen included negative blood cultures, negative serology for toxoplasmosis and brucella but an increased anti-streptolysin (ASO) titre of 500 Todd units.

The initial diagnosis was considered to be rheumatic fever. However, despite treatment for two to three weeks with salicylates and benzyl penicillin his symptoms including fever continued unabated. A diagnosis of systemic onset juvenile chronic arthritis (SOJCA) was made and he was given prednisolone 60 mg/day (approximately 1 mg/kg). This led to a rapid improvement in his joint symptoms and resolution of his rash.

His systemic features remained problematic over the next few months reappearing with attempted reductions in his corticosteroid dose. However, they eventually settled by July 1983 and he was maintained with non-steroidal agents …