A case of simultaneous, biopsy-proven, classic, ANCA-positive Wegener's granulomatosis and anti-GBM disease, but without detectible circulating anti-GBM antibodies

Aleksandra Gmurczyk*, Shubhada N. Ahya, Robert Goldschmidt, George Kim, L. Tammy Ho, Kevin Nash

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

Wegener's granulomatosis (WG) is a systemic, necrotizing, granulomatous vasculitis of unknown etiology. Approximately 75% of cases present as classic WG with both pulmonary and renal involvement, while the remaining 25% of patients present with a limited form with either predominantly upper or lower respiratory tract symptoms. Ninety percent of WG patients have circulating anti-neutrophil cytoplasmic antibodies (ANCA), and approximately 10% have both circulating ANCA antibodies and concomitant anti-glomerular basement membrane (anti-GBM) disease on renal biopsy. Virtually all of these patients also have circulating anti-GBM antibodies. While it has been reported that some patients with ANCA vasculitis have circulating anti-GBM antibodies, and patients with anti-GBM disease may have positive ANCA, review of the literature does not demonstrate other cases of biopsy-proven, simultaneous, ANCA-associated vasculitis and anti-GBM disease. We report a case of simultaneous, biopsy-proven, classic, ANCA-positive WG and anti-GBM disease, but without detectible circulating anti-GBM antibodies. We present findings characteristic of both WG and linear IgG deposition along the GBM suggesting concurrent anti-GBM disease, in the absence of detectable circulating anti-GBM antibodies. Possible theories to explain the absence of these antibodies are discussed.

Original languageEnglish (US)
Pages (from-to)1078-1083
Number of pages6
JournalTheScientificWorldJournal
Volume10
DOIs
StatePublished - Jun 15 2010

Keywords

  • ANCA
  • Anti-GBM antibody
  • Kidney injury
  • Wegener's granulomatosis

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Environmental Science(all)

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