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The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis.
Nasr, Samih H; Collins, A Bernard; Alexander, Mariam Priya; Schraith, Daniel F; Herrera Hernandez, Loren; Fidler, Mary E; Sethi, Sanjeev; Leung, Nelson; Fervenza, Fernando C; Cornell, Lynn D.
Afiliação
  • Nasr SH; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
  • Collins AB; Pathology Service, Massachusetts General Hospital, Boston, Massachusetts, USA.
  • Alexander MP; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
  • Schraith DF; Department of Pathology and Laboratory Medicine, Gundersen Health System, La Crosse, Wisconsin, USA.
  • Herrera Hernandez L; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
  • Fidler ME; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
  • Sethi S; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
  • Leung N; Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA.
  • Fervenza FC; Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA.
  • Cornell LD; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: cornell.lynn@mayo.edu.
Kidney Int ; 89(4): 897-908, 2016 Apr.
Article em En | MEDLINE | ID: mdl-26994577
ABSTRACT
Classic anti-glomerular basement membrane (GBM) disease presents with rapidly progressive glomerulonephritis (GN) with or without pulmonary hemorrhage. On biopsy typical disease displays bright polytypic linear GBM staining for IgG by immunofluorescence and diffuse crescentic/necrotizing GN on light microscopy. Here, we studied 20 patients with atypical anti-GBM nephritis typified by bright linear GBM staining for immunoglobulins but without a diffuse crescentic phenotype. Patients had hematuria, proteinuria, and mild renal insufficiency, without pulmonary hemorrhage. Light microscopy showed endocapillary proliferative GN in 9 patients, mesangial proliferative GN in 6, membranoproliferative GN in 3, and focal segmental glomerulosclerosis with mesangial hypercellularity in 2. Eight of the 20 showed features of microangiopathy. Crescents/necrosis were absent in 12 and were focal in 8 patients. Bright linear GBM staining for IgG was seen in 17 patients, IgM in 2, and IgA in 1 patient, which was polytypic in 10 patients and monotypic in 10 patients. No circulating α3NC1 antibodies were detected by commercial ELISA. The 1-year patient and renal survival rates were 93% and 85%, respectively. Thus, atypical anti-GBM nephritis is a rare variant of anti-GBM disease characterized clinically by an indolent course, no pulmonary involvement, and undetectable circulating α3NC1 antibodies. Further studies are needed to characterize the molecular architecture of GBM autoantigens in these patients and establish optimal therapy.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doença Antimembrana Basal Glomerular / Rim Limite: Adolescent / Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2016 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Doença Antimembrana Basal Glomerular / Rim Limite: Adolescent / Adult / Aged / Aged80 / Female / Humans / Male / Middle aged Idioma: En Ano de publicação: 2016 Tipo de documento: Article