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),
hypoproteinemia, 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
Goodpasture'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.