The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition

John L. Niles, Erwin P. Böttinger, Guillermo R. Saurina, K. J. Kelly, Guo Li Pan, A. Bernard Collins, Robert T. McCluskey

Research output: Contribution to journalArticlepeer-review

194 Scopus citations

Abstract

Background: In the absence of evidence of arteritis or Wegener's granulomatosis, the syndrome of lung hemorrhage and nephritis has been commonly associated with anti-glomerular basement membrane (GBM) antibodies. However, it has been increasingly recognized that many cases are associated with antineutrophil cytoplasmic antibodies (ANCAs). Objective: To review available clinical and pathologic findings to determine the diseases accounting for lung hemorrhage and nephritis. Methods: We studied the records of 750 patients from whom serum samples were sent to our laboratory for anti-GBM antibody assays between 1981 and 1993 and found 88 patients with evidence of lung hemorrhage and nephritis. Serum samples were retested, using current methods, for anti-GBM antibodies (against noncollagenous 1 domain of the α3 chain of type IV collagen) and for antibodies to proteinase 3 and myeloperoxidase - the two types of ANCA of diagnostic value. Results: Of 88 patients with evidence of lung hemorrhage and nephritis, 48 had ANCAs, six had anti-GBM antibodies, and seven had both. In 48 patients with ANCAs, the pathologic findings that accounted for the pulmonary renal syndrome were pauci-immune necrotizing and crescentic glomerulonephritis and pulmonary capillaritis. Only eight had convincing evidence (during life) of Wegener's granulomatosis and only one other had documented arteritis. In 27 patients without ANCAs or anti-GBM antibodies, a variety of unrelated renal and pulmonary diseases were found. Conclusions: The largest group of patients who present with the syndrome of lung hemorrhage and nephritis have ANCAs and not anti-GBM antibodies. Appropriate tests for antibodies to proteinase 3, antibodies to myeloperoxidase, and anti-GBM antibodies provide reliable guides for making a diagnosis in patients with this pulmonary renal syndrome.

Original languageEnglish (US)
Pages (from-to)440-445
Number of pages6
JournalArchives of Internal Medicine
Volume156
Issue number4
DOIs
StatePublished - Feb 26 1996
Externally publishedYes

ASJC Scopus subject areas

  • Internal Medicine

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