title: A case of a 6-year-old girl with anti-neutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis.
abstract: A 6-year-old girl was admitted to our hospital with proteinuria, hematuria, skin rash and joint pain of the lower limbs. Due to rapid progression of renal insufficiency, hemodialysis and peritoneal dialysis were performed. She was diagnosed with rapidly progressive glomerulonephritis. Kidney biopsy showed severe crescent formation (50% of glomeruli) and no deposition of any immunoglobulins or complements. Serologically, anti-neutrophil cytoplasmic autoantibody (ANCA) was negative not only by ELISA against proteinase-3 and myeloperoxidase-ANCA but also by indirect immunofluorescent assay against cytoplasmic and perinuclear ANCA. Anti-glomerular basement membrane antibody was also negative. In the acute phase, proinflammatory cytokines such as soluble tumor necrosis factor receptor 1 (sTNFR1), soluble interleukin (IL)-2 receptor (sIL2R), IL-6 and chemokine IL-8 were elevated. The patient was diagnosed with ANCA-negative pauci-immune crescentic glomerulonephritis (CrGN). Intensive treatment with methylprednisolone pulse therapy, plasma exchange, and multiple drug therapy including prednisolone and cyclophosphamide resulted in histopathological improvement and complete remission of proteinuria. There was a possibility that sTNFR1, sIL2R, IL-6 and IL-8 might be involved in the initiation and progression of ANCA-negative pauci-immune CrGN, and to remove and suppress these cytokines might be an effective way to treat ANCA-negative pauci-immune CrGN.
authors.affiliation: Department of Pediatrics, Takamatsu Red Cross Hospital, 4-1-3 Ban-cho, Takamatsu, Kagawa 760-0017, Japan. maki.shmz@mac.com
authors.forename: Maki
authors.initials: M
authors.lastname: Shimizu
authors.forename: Takanori
authors.initials: T
authors.lastname: Sekiguchi
authors.forename: Natsuko
authors.initials: N
authors.lastname: Kishi
authors.forename: Aya
authors.initials: A
authors.lastname: Goji
authors.forename: Tomoko
authors.initials: T
authors.lastname: Takahashi
authors.forename: Hiroko
authors.initials: H
authors.lastname: Kozan
authors.forename: Zenichi
authors.initials: Z
authors.lastname: Sakaguchi
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Kinoshita
authors.forename: Sato
authors.initials: S
authors.lastname: Matsuura
authors.forename: Kenichi
authors.initials: K
authors.lastname: Suga
authors.forename: Maki
authors.initials: M
authors.lastname: Urushihara
authors.forename: Shuji
authors.initials: S
authors.lastname: Kondo
authors.forename: Shoji
authors.initials: S
authors.lastname: Kagami
authors.forename: Katsuaki
authors.initials: K
authors.lastname: Ohara
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D001323:Autoantibodies / Q000032:analysis; D002648:Child; D003520:Cyclophosphamide / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D005921:Glomerulonephritis / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D008775:Methylprednisolone / Q000008:administration & dosage; D010956:Plasmapheresis; D011239:Prednisolone / Q000008:administration & dosage; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology; D020551:Pulse Therapy, Drug
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; C051951:antiglomerular basement membrane antibody; D003520:Cyclophosphamide; D011239:Prednisolone; D008775:Methylprednisolone