Wegener granulomatosis in pediatric patients Wegener granulomatosis is more easily recognized as a distincl clinical entity than other vasculitides because the initial clinical features frequently include granulomatous vasculitis of the upper and lower respiratory tract and glomerulonephritis. Although the diseasd has been lethal in the past, prolonged survival and avoidance of end-stage kidney disease can now be expected when . cyclophosphamide therapy is introduced early in the course. We report four children with Wegener granulomatosis in whom the initial elinical findings suggested Henoch-Schi~nlein purpura. In two of the patients Wegener granulomatosis was not recognized until after end-stage kidney disease had developed. The course in these patients emphasizes the need for attention to even scant evidence of inflammation of the upper or lower respiratory tract in patients with glomerulonephritis. Appropriate diagnostic studies may then lead to recognition of Wegener granulomatosis and the prompt institution of appropriate treatment. (J PEDIATR 106:739, 1985) Sue L. Hail, M.D., Laurie C. Miller, M.D., Eileen Duggan, M.D., S. Michael Mauer, M.D., Eugene C. Beatty, M.D., and Stanley Hellerstein, M.D. Kansas City, Missouri, and Minneapolis, Minnesota WEGENER GRANULOMATOSIS is characterized by nec- rotizing granulomatous vasculitis of the upper and lower respiratory tract and glomerulonephritis. Although this is the classic triad, there is frequently a disseminated vascu- litis involving other small arteries and veins that may lead to involvement of any organ and a variety of clinical findings. ~ The disease is uncommon in children, with only 27 cases reported in the pediatric age group. 2'5 Because prolonged survival can now be expected with cyclophos- phamide treatment, a specific diagnosis is urgent in this previously lethal disease. We report four children with Wegener granulomatosis in whom the initial clinical fea- tures resembled those of Henoch-SchSnlein purpura, illus- trating that the diagnosis of Wegener granulomatosis in children may be elusive because of atypical presentation and stow evolution of the disease. CASE REPORTS Patient 1. This white girl had no serious health problems until she was hospitalized at age 13 years because of severe abdominal From the Departments of Pediatrics, University of Missouri-- Kansas City, and the University of Minnesota Medical School. Submitted for publication March 9, 1984; accepted Oct. 5, 1984. Reprint requests: Stanley Hellerstein, M.D., Section of Pediatric NephroIogy, The Children's Mercy Hospital, 24th at GilIham Road. Kansas City, MO 64108. pain with nausea and vomiting. Physical findings included con- junctivitis of the left eye and pain on palpation of the epigastrium. Findings of an upper gastrointestinal tract x-ray study were suggestive of a small posterior duodenal ulcer, and the patient was given antacids and cimetidine. There was mild anemia, marked leukocytosis, and eosinophilia in the range of 10% to 20%. Hematuria, proteinuria, and pyuria were present. The serum creatinine concentration was 1.0 mg/dl. Serum complement levels were normal, and antinuclear antibody titer and LE cell prepara- tion were negative. The patient was transferred to The Children's Mercy Hospital. On admission the significant physical findings were pallor and bilateral conjunctivitis. Hematuria and proteinuria were present. The centrifuged urinary sediment showed innumerable red blood cells and 0 to 4 granular and cellular casts per high-power field. On the third hospital day two small areas of petechiae and purpura were noted over the lateral malleoli. Over the next 3 to 4 days a purpuric rash developed over both lower extremities and the buttocks. Many of the lesions were palpable and thought to be infiltrates. Arthralgia and mild arthritis developed, but the abdominal symptoms subsided. Salicylate therapy was started. About 24 hours later the patient again developed severe abdomi- nal pain and vomiting. An upper gastrointestinal tract series showed prominent xnticosal folds with spiculated changes and thumb print deformitieL This was interpreted as a vasculitis, compatible with Henoch-SchSnlein purpura. Th~ere was dramatic resolution of the gastrointestinal tract symptoms within 24 hours of starting treatment with intravenously administered hydrocorti- sone. The Journal of P E D l A T R I C S 739