title: Kidney transplantation for a patient with refractory childhood-onset ANCA-associated vasculitis.
abstract: A 14-year-old Japanese girl was admitted to our institution for the evaluation of renal dysfunction. Her serum creatinine was 1.1 mg/dL, proteinuria was 1.5 g/day, the urine sediment contained numerous erythrocytes per high-power field, and she was positive for myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA). Proteinuria was first noted at the age of 12 years. Renal biopsy showed crescentic glomerulonephritis with slight immunoglobulin A (IgA) deposition. A diagnosis of ANCA-associated vasculitis was made. Immunosuppressive therapy was initiated, including steroid pulse therapy and intravenous cyclophosphamide pulse therapy, but hemodialysis was required after 6 years. Eight months after the patient became anuric and her MPO-ANCA titer became negative, living-related donor kidney transplantation was done from her mother. ANCA became slightly positive 2 years later, but the patient remains stable without proteinuria or hematuria at 4 years after surgery. This case suggests that kidney transplantation can be performed successfully for a patient with refractory childhood-onset ANCA-associated vasculitis, and that remission of vasculitis associated with ANCA negativity at transplantation may contribute to a better renal prognosis in this patient.
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Kaseda
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Marui
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suwabe
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Junichi
authors.initials: J
authors.lastname: Hoshino
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Keiichi
authors.initials: K
authors.lastname: Sumida
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Hayami
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Koki
authors.initials: K
authors.lastname: Mise
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Kiho
authors.initials: K
authors.lastname: Tanaka
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.|c Okinaka Memorial Institute for Medical Research, Toranomon Hospital , Tokyo , Japan.
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Tomikawa
authors.affiliation: b Department of Pathology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Fujii
authors.affiliation: b Department of Pathology , Toranomon Hospital , Tokyo , Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ohashi
authors.affiliation: a Nephrology Center, Toranomon Hospital , Tokyo , Japan.|c Okinaka Memorial Institute for Medical Research, Toranomon Hospital , Tokyo , Japan.
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Ubara
mesh_terms: D000293:Adolescent; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology / Q000601:surgery*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D005260:Female; D006801:Humans; D007677:Kidney Function Tests; D016030:Kidney Transplantation; D009195:Peroxidase / Q000276:immunology; D011507:Proteinuria / Q000276:immunology / Q000601:surgery*; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase
keywords: ANCA-associated vasculitis; anti-neutrophil cytoplasmic antibody; childhood onset; kidney transplantation; microscopic polyangiitis