Lupus nephropathy and cardiopulmonary and hepatic dysfunctions in a child.

Systemic lupus erythematosus (SLE) is a potentially fatal autoimmune multi-systemic rheumatologic disorder. An unusual case is reported of an 11.9-year-old Nigerian girl who was diagnosed after 2.8 years of non-specific symptoms and six episodes of recurrent haemolysis and pancytopaenia warranting blood transfusions. At diagnosis, she had hepatitis, polyarthritis, nephropathy, and cardiopulmonary and bone-marrow dysfunctions. Lymphopaenia, thrombocytopaenia, and direct antiglobulin-test positive haemolytic anaemia were present. Rapid resolution of disease activity followed exchange blood transfusion after an initial poor response to corticosteroid and cyclophosphamide therapy. Any child with recurrent haemolysis and pancytopaenia of unknown aetiology should be investigated for SLE.