Diagnostic and management problems in a complex case of connective tissue disease

A 28-year-old Nigerian woman presented with persistent pyrexia, marked pruritus, eosinophilia, myalgias, flitting arthralgias, serositis and massive splenomegaly. Intensive investigation for an infective or neoplastic aetiology proved negative. Empirical treatment for helminthic infections and tuberculosis was unhelpful. Although there were no specific clues to suggest an underlying connective tissue disease, a trial of steriods and azathioprine was introduced, with no obvious response. Her condition deteriorated to a point where it was decided that intravenous immunosuppressive therapy was needed and subsequently, her condition improved remarkably. This patient illustrates the problems in the diagnosis and management of complex disorders, particularly when classical tests for connective tissue diseases are absent. Also, we would like to report that marked pruritus can be associated with connective tissue disease.</p