The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis

Samih H. Nasr, A. Bernard Collins, Mariam P Alexander, Daniel F. Schraith, Loren Herrera Hernandez, Mary E. Fidler, Sanjeev M Sethi, Nelson Leung, Fernando Custodio Fervenza, Lynn D. Cornell

Research output: Contribution to journalArticle

27 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)897-908
Number of pages12
JournalKidney International
Volume89
Issue number4
DOIs
StatePublished - Apr 1 2016

Fingerprint

Glomerular Basement Membrane
Nephritis
Glomerulonephritis
Anti-Glomerular Basement Membrane Disease
Staining and Labeling
Lung
Microscopy
Immunoglobulin G
Hemorrhage
Membranoproliferative Glomerulonephritis
Light
Focal Segmental Glomerulosclerosis
Antibodies
Autoantigens
Hematuria
Proteinuria
Immunoglobulin A
Renal Insufficiency
Fluorescent Antibody Technique
Immunoglobulin M

Keywords

  • anti-GBM disease
  • glomerulonephritis
  • kidney biopsy
  • nephrotic syndrome
  • renal pathology

ASJC Scopus subject areas

  • Nephrology

Cite this

Nasr, S. H., Collins, A. B., Alexander, M. P., Schraith, D. F., Herrera Hernandez, L., Fidler, M. E., ... Cornell, L. D. (2016). The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis. Kidney International, 89(4), 897-908. https://doi.org/10.1016/j.kint.2016.02.001

The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis. / Nasr, Samih H.; Collins, A. Bernard; Alexander, Mariam P; Schraith, Daniel F.; Herrera Hernandez, Loren; Fidler, Mary E.; Sethi, Sanjeev M; Leung, Nelson; Fervenza, Fernando Custodio; Cornell, Lynn D.

In: Kidney International, Vol. 89, No. 4, 01.04.2016, p. 897-908.

Research output: Contribution to journalArticle

Nasr, Samih H. ; Collins, A. Bernard ; Alexander, Mariam P ; Schraith, Daniel F. ; Herrera Hernandez, Loren ; Fidler, Mary E. ; Sethi, Sanjeev M ; Leung, Nelson ; Fervenza, Fernando Custodio ; Cornell, Lynn D. / The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis. In: Kidney International. 2016 ; Vol. 89, No. 4. pp. 897-908.
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