[Goodpasture's syndrome (author's transl)].
Goodpasture's syndrome occurred in six patients (four men and two women) aged 18-32 years. Haemoptysis, anaemia and abnormal urinary findings with erythrocyturia were the first signs of the disease in five instances, in one the renal signs predominated. In five patients the disease took a malignant course with uraemia developing within six weeks. One patient is in a spontaneous remission eleven months after the onset of the disease. Three patients died of recurrent haemoptysis (non-nephrectomised), two nephrectomised patients are still alive half a year and three and a half years later, respectively. A typical linear IgG-, beta1A, alpha2D and beta1E deposition along the glomerular capillaries was demonstrated immunohistologically in all subjects. Of three lung biopsies one also had typical IgG and beta1A-positive linear fluorescence along the alveolar basal membrane. Immunoglobulins with basal membrane antibody activity were eluted from renal homogenates in two patients, basal membrane antibody activity in serum was demonstrated only in one of three patients.
D000293:Adolescent; D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications / Q000175:diagnosis* / Q000276:immunology / Q000601:surgery; D001485:Basement Membrane; D001706:Biopsy; D002196:Capillaries; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007678:Kidney Glomerulus / Q000276:immunology; D008168:Lung / Q000276:immunology; D008297:Male; D009392:Nephrectomy; D012075:Remission, Spontaneous; D013997:Time Factors; D014511:Uremia / Q000150:complications
D007136:Immunoglobulins
Klinik und immunologie des goodpasture-syndroms