A boy with consecutive development of SLE and Wegener granulomatosis

Abstract

An 11-year-old boy with consecutive development of systemic lupus erythematosus (SLE) and Wegener granulomatosis (WG) is presented. He was first admitted to the hospital with the findings of SLE, including crescentic glomerulonephritis, Coombs' test-positive hemolytic anemia, hypocomplementemia, antinuclear antibody (ANA) positivity, and elevated levels of anti-double-stranded (ds) DNA antibodies. He was treated successfully with steroids, cyclophosphamide, and peritoneal dialysis. One month after his discharge he developed an apparent viral infection. Three weeks afterwards he was readmitted with the findings of lower respiratory tract involvement, maxillary sinusitis, nasal septum perforation, p- and c-antineutrophil cytoplasmic antibody (ANCA) positivity, but normal complement, ANA, and anti-ds DNA levels, suggesting the diagnosis of WG. He did not respond to anti-infectious and immunosuppressive treatment, and he died of Pseudomonas sepsis.