Fatal relapse in Goodpasture's syndrome 3 years after plasma exchange

Since 1919 about 400 cases with Goodpasture's syndrome have been published, and since 1975 about 100 of these patients were treated with plasma exchange in addition to immunosuppression. A remission was achieved in 50% of the 100 cases, and in 7 a relapse was reported. In this paper, we are reporting a case with a fatal relapse 3 years after successful plasma exchange treatment. This patient was a smoker, she had been exposed to hydrocarbon solvents, and she had the HLA tissue antigens DR2 and MT3. Lung biopsy by fiberoptic bronchoscopy revealed linear staining of IgG at the alveolar basement membrane. During relapse pulmonary infiltrations and macrohematuria did not respond a second time to methylprednisolone pulses nor to plasma exchange treatment and the patient died of massive lung hemorrhage.