Case report
Goodpasture's syndrome with normal renal function

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Abstract

Two male patients with Goodpasture's syndrome manifesting as severe pulmonary hemorrhage with minimal renal abnormalities are described. Both patients had microscopic hematuria with normal renal function, and one had transient proteinuria. Renal glomerular histology was normal and electron microscopic findings revealed no electron-dense deposits, but immunofluorescence of immunoglobulin G (IgG) was positive in a linear fashion along glomerular capillary basement membranes in both patients. Pulmonary hemorrhage was arrested following prednisone therapy, and both patients have normal pulmonary and renal function at five and 13 months of follow-up. The literature on patients with Goodpasture's syndrome, pulmonary hemorrhage and normal renal function with minimal proteinuria is reviewed. It is suggested that a subset of patients with Goodpasture's sybdrome have pulmonary hemorrhage as their major manifestation. Since prednisone seems to have an apparent beneficial effect on pulmonary hemorrhage, and relatively good prognosis, this diagnosis should be considered in patients with idiopathic pulmonary hemorrhage.

References (26)

  • GJ Beirne et al.

    Immunohistology of the lung in Goodpasture's syndrome

    Ann Intern Med

    (1968)
  • PJ Dahlberg et al.

    Abstracts, American Society of Nephrology

    (1977)
  • CS MacGregor et al.

    Fatal nephritis complicating idiopathic pulmonary hemosiderosis in young adults

    Thorax

    (1960)
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    1

    From the Departments of Medicine, Pathology and Anesthesiology, University of Wisconsin Center for Health Sciences, Madison, Wisconsin.

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