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Goodpasture syndrome involving overlap with Wegener's granulomatosis and anti-glomerular basement membrane disease

  • Raghu Kalluri(corresponding author)
    ,
  • Kevin Meyers
    ,
  • Andras Mogyorosi
    ,
  • Michael P. Madaio
    ,
  • Eric G. Neilson
*Corresponding author for this work
  • Harvard University
    ,
  • University of Pennsylvania
Scholary Output:
Contribution to journal
Article
Peer-review

Open access

Abstract

A 68-year-old Caucasian woman presented to the hospital with nodular pulmonary infiltrates and acute renal failure. Wegener's granulomatosis was initially considered to be most likely because of the presence of increased serum levels of c-anti-neutrophil cytoplasmic antibodies (c-ANCA). A consultation through the Internet after a renal biopsy demonstrated crescentic, necrotizing glomerulonephritis and linear deposits of immunoglobulin G (IgG) and complement C3, typical of anti-glomerular basement membrane (GBM) disease. Hemodialysis was instituted; however, the patient suddenly developed a massive cerebral hemorrhage and died before full therapy could take effect. Postmortem analysis of the patient's sera revealed high titers of IgG against the α3 NC1 domain of type IV collagen. Serologic evidence of both p-ANCA and anti-GBM antibodies are becoming more frequently recognized in the setting of rapidly progressive glomerulonephritis. The patient reported here had the unusual combination of c-ANCA antibodies with anti-GBM disease, and this association raises complex questions regarding the pathogenesis of this type of renal injury.

Publication Information

Output type

Scholary Output:
Contribution to journal
Article
Peer-review

Original language

English (US)

Pages from-to (Number of pages)

Pages 1795-1800 (6 pages)

Journal (Volume, Issue Number)

Journal of the American Society of Nephrology (Volume 8, Issue 11)

Publication milestones

  • Published - 11/1997

Publication status

Published - 11/1997

ISSN

1046-6673

Publication IDs

  • Scopus: 0030716935
  • PubMed: 9355084

Publication metrics

Metrics

SciVal
FWCI
0.70
SciVal
Author count
5
SciVal
citations
53
SciVal
Paper percentile
88
Scopus
citations
Fractional count
1
Fractional count
0.20
Fractional count
4
Fractional count
0.80
Fractional count
1
Fractional count
1

PlumX, opens in new tab

Captures
22
Citation count
65

Funding Details

FunderFunding number
NIDDK
R01DK030280