Pulmonary-renal syndrome: clinical similarity amidst etiologic diversity

The medical records and morphologic materials (59 biopsies, 7 autopsies) of 21 patients presenting with a pulmonary-renal syndrome (PRS) were reviewed. A variety of disorders (Goodpasture's syndrome, 3; vasculitis, 7; idiopathic crescentic glomerulonephritis, 7; spurious PRS, 4) may have PRS presentation. Seventeen patients had extrarenal vasculitis and/or crescentic glomerulonephritis and were treated with immunosuppression. Seven patients died, six from complications related to infections and cardiovascular disease but only one from his original disease. Four other patients ("spurious" PRS) who did not have crescents or extrarenal vasculitis did well with supportive therapy alone. We conclude that a pulmonary-renal syndrome is a common manifestation of diverse disorders which differ in their prognosis and therapy. Since clinical parameters alone are too insensitive to resolve the etiologic possibilities, multiple biopsies and serologies tests are required for a reliable diagnosis.