Antiphospholipid antibodies in patients with scleroderma: prevalence and clinical significance

A 14 year old girl presented with a year's history of joint pain, restriction, and swelling, initially diagnosed as juvenile idiopathic arthritis. She had no past medical problems but had given up her leisure pursuits owing to disability. Examination disclosed non-pitting oedema to midcalf; a woody texture of the soft tissues of the forearms and calves with fixed flexion deformities of her fingers, wrists, knees, and ankles due to the soft tissue changes, and normal overlying skin. Investigations showed the following results: normal muscle enzymes, eosinophilia of 1.7610 9 /l, erythrocyte sedimentation rate 41 mm/1st h, haemoglobin 112 g/l, negative autoantibodies, and normal complement levels. The differential diagnosis included eosinophilic fasciitis (EF) and scleroderma. At this time, the patient requested counselling as she had difficulty accepting an initial misdiagnosis, delayed correct diagnosis, and the uncertainty of the prognosis.