A boy with consecutive development of SLE and Wegener granulomatosis

Byline: Ozlem Erdogan (1), AyAe Aner (1,5), Gulay Demircin (1), Mehmet Bulbul (1), Leyla MemiA (2), Cigdem Uner (3), Nural Kiper (4) Keywords: Overlap syndrome; Systemic lupus erythematosus; 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. Author Affiliation: (1) Department of Pediatric Nephrology, Dr. Sami Ulus Children's Hospital, Ankara, Turkey (2) Department of Pathology, University Hospital of Gazi, Ankara, Turkey (3) Department of Radiology, Dr. Sami Ulus Children's Hospital, Ankara, Turkey (4) Department of Pediatric Chest Disease, University Hospital of Hacettepe, Ankara, Turkey (5) Bilkent 1, CamlA+-k Sitesi, D7-Blok, no 21, 06530 Ankara, Turkey Article History: Registration Date: 23/10/2003 Received Date: 06/01/2003 Accepted Date: 08/10/2003 Online Date: 23/01/2004