A case of Goodpasture's syndrome with massive pulmonary hemorrhage ameliorated by cyclophosphamide pulse therapy

Abstract

A 22-year-old woman was admitted to our hospital for evaluation of fever, renal dysfunction, and a 3-month-history of macrohematuria . Laboratory evaluation revealed proteinuria (1.8 g/day), hypo proteinemia, microcytic microchromic anemia, renal failure (blood urea nitrogen 30.3 mg/dl, serum creatinine 4.0 mg/dl), and positive serum antiglomerular basement membrane (anti-GBM) antibody . Renal biopsy revealed cellular crescents in all 8 glomeruli and partial rupture of the GBM. The interstitium showed severe inflammatory cell infiltration . Immunofluorescent examination revealed linear deposits of IgG and C3 along the GBM . Pulmonary biopsy revealed linear deposits of IgG along the alveolar basement membrane in the Immunofluorescent examination . A diagnosis of Goodpas ture's syndrome was made because all of the diagnostic criteria were fulfilled . After admission, the patient's renal function deteriorated rapidly . Hemodialysis was started, and the patient was treated with methylprednisolone pulse therapy and oral prednisolone with double filtration plasma pheresis (DFPP) . However, her renal function did not improve. On the 30th hospital day, she showed hemoptysis, and a chest X-ray and CT revealed massive bilateral pulmonary hemorrhage . Despite treatment with pulsed methylprednisolone, oral prednisolone(80 mg/day), and DFPP, the pulmonary hemorrhage improved only transiently, worsening again 5 days later . Cyclophosphamide pulse therapy was administered. After this treatment, the patient's pulmonary manifestations and pulmonary hemorrhage improved. At the present time she is on maintenance dialysis therapy without pulmonary manifestations . These findings suggest that cyclophosphamide pulse therapy is effective against Goodpasture's syndrome with massive pulmonary hemorrhage showing resistance to other conventional therapy .