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Variant "Goodpasture's Syndrome"?: The Need for Immunologic Criteria in Rapidly Progressive Glomerulonephritis and Hemorrhagic Pneumonitis

[+] Article, Author, and Disclosure Information

Supported in part by grants HD03381-03 and AI00013-12, National Institutes of Health, Bethesda, Md. Dr. Kohler is a recipient of a Research Career Development Award, HD 42,404-02, National Institute of Child Health and Human Development, National Institutes of Health.

▸Requests for reprints should be addressed to Peter F. Kohler, M.D., University of Colorado Medical Center, Denver, Colo. 80220

Ann Intern Med. 1971;75(1):67-76. doi:10.7326/0003-4819-75-1-67
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The immunopathogenic mechanisms in three men with rapidly progressive glomerulonephritis and hemorrhagic pneumonitis were studied. Nephritis and fatal pulmonary hemorrhage occurred simultaneously in two patients. Typical Goodpasture's syndrome was defined in one by the presence of noncomplement-fixing IgG antiglomerular basement membrane (GBM) antibodies in his circulation, kidney, and lung. Immunofluorescent studies were negative in the second patient, although an immune complex pathogenesis was suggested by the presence of mixed cryoglobulinemia, decreased serum complement, and arteritis. The third patient also had anti-GBM-mediated nephritis, but his ultimately fatal pulmonary disease did not develop until after nephrectomy. Recurrence of the same anti-GBM nephritis was documented in his renal transplant at necropsy. It is suggested that "Goodpasture's syndrome" be restricted to patients with anti-GBM antibody-mediated nephritis and concomitant hemorrhagic pneumonitis.





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