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The Syndrome of Lung Hemorrhage and Nephritis Is Usually an ANCA-Associated Condition
John L. Niles, MD;
Erwin P. Böttinger, MD;
Guillermo R. Saurina, MD;
K. J. Kelly, MD;
GuoLi Pan, MD;
A. Bernard Collins;
Robert T. McCluskey, MD
Arch Intern Med. 1996;156(4):440-445.
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 antiGBM 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  3 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.
(Arch Intern Med. 1996;156:440-445)
Author Affiliations
From the Departments of Medicine (Drs Niles, Saurina, and Kelly) and Pathology (Drs Niles, McCluskey, and Pan and Mr Collins), Massachusetts General Hospital, Boston, and the National Cancer Institute, Bethesda, Md (Dr Böttinger).
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