Systemic lupus erythematosus in a child with sickle cell disease

Although sickle cell disease (SCD) and systemic lupus erythematosus (SLE) are two distinct chronic diseases, many clinical features are common to both conditions. We describe a young patient who had a mild clinical course of SCD until SLE developed when he was 15 years old. His initial manifestations of SLE including fever, chest pain, and lung infiltration with pleural effusion were thought to be complications of SCD. However, a deteriorating clinical course, presence of facial and truncal rash, and persistent pleural effusion led to the diagnosis of SLE. We compare our case and the 10 previously reported cases and discuss the possible association of complement defects and the pathogenesis of SLE in patients with SCD. Our report illustrates the importance of considering other disease processes when clinical features are atypical of SCD.