title: Renal-limited necrotizing granulomatous vasculitis in a pediatric patient.
abstract: A 10-year-old girl presented with mild proteinuria and hypertension. Laboratory data indicated slightly elevated serum creatinine (0.67 mg/dL) and elevated serum IgG (2111 mg/dL). On renal arteriography mild stenosis over the entire length of the right renal artery and irregular stenosis of the interlobar arteries in the right kidney were seen. She was diagnosed with renovascular hypertension, and received conventional anti-hypertensive therapy, but did not respond to them. The right kidney had atrophy and dysfunction on technetium-99m-labeled dimercaptosuccinic acid renal scintigraphy, and was therefore resected. Histopathology of the kidney indicated severe necrotizing granulomatous vasculitis affecting the arteries from the renal hilus to the interlobar area. After nephrectomy plus steroid pulse therapy, blood pressure and urinary protein returned to normal. To our knowledge, this is the first report of necrotizing granulomatous vasculitis limited to the medium-sized renal arteries.
authors: Department of Pediatrics, Jichi Children's Medical Center Tochigi, Jichi Medical University, Tochigi.
authors: Jun
authors: J
authors: Odaka
authors: Department of Pediatrics, Jichi Children's Medical Center Tochigi, Jichi Medical University, Tochigi.
authors: Takahiro
authors: T
authors: Kanai
authors: Department of Pediatrics, Jichi Children's Medical Center Tochigi, Jichi Medical University, Tochigi.
authors: Takane
authors: T
authors: Ito
authors: Department of Pediatrics, Jichi Children's Medical Center Tochigi, Jichi Medical University, Tochigi.
authors: Takashi
authors: T
authors: Saito
authors: Department of Pediatrics, Jichi Children's Medical Center Tochigi, Jichi Medical University, Tochigi.
authors: Jun
authors: J
authors: Aoyagi
authors: Department of Pathology, Koshigaya Hospital, Dokkyo Medical University, Saitama, Japan.
authors: Yoshihiko
authors: Y
authors: Ueda
authors: Department of Pediatrics, Jichi Children's Medical Center Tochigi, Jichi Medical University, Tochigi.
authors: Takanori
authors: T
authors: Yamagata
mesh_terms: D000792:Angiography; D000959:Antihypertensive Agents / Q000627:therapeutic use; D002648:Child; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000000981:diagnostic imaging* / Q000188:drug therapy; D006801:Humans; D006978:Hypertension, Renovascular / Q000000981:diagnostic imaging* / Q000188:drug therapy; D007674:Kidney Diseases / Q000000981:diagnostic imaging* / Q000188:drug therapy; D009392:Nephrectomy; D011877:Radionuclide Imaging; D019783:Technetium Tc 99m Dimercaptosuccinic Acid
chemical_list: D000959:Antihypertensive Agents; D005938:Glucocorticoids; D019783:Technetium Tc 99m Dimercaptosuccinic Acid
keywords: child; hypertension; proteinuria; renal artery; vasculitis