[Current aspects of Goodpasture's syndrome].
Goodpasture's syndrome is an auto-immune disorder with antibodies directed against alveolar and glomerular basement membrane. These antibodies are usually present in the serum but their detection is difficult, requiring a radio-immunological technique which remains uncommon. The fixed antibodies are detected more easily and more constantly in the kidney tissue than in the lung; they appear on a kidney biopsy in the form of linear deposits of immunoglobulins on the basement membrane. Thus, the renal biopsy appears to be the essential requirement to establish a diagnosis of Goodpasture's syndrome, and one should not hesitate to perform a biopsy in the presence of diffuse alveolar haemorrhage without an evident aetiology and even in the absence of any biological evidence of renal disease. Alveolar lavage may be used to confirm a haemorrhage which has been purely alveolar. The treatment for respiratory and renal recovery, using immunological therapy includes corticosteroids, cytotoxic drugs and plasmapheresis. The exact methods are not yet fully defined during the course on certain clinical forms of the disease: for oliguric patients, dialysis is the single treatment; the isolated pulmonary forms have often been treated and improved by corticotherapy alone. The responsible antigen, probably unique, has been recently been identified as collagen type IV of the glomerular basement membrane. The pathogenesis of Goodpasture's syndrome remains unknown. The inhalation of hydrocarbons has been frequently noted. The dismal prognosis in the natural history of the disease and the therapeutic possibilities available make diagnosis an urgency. Currently, renal biopsy represents the most reliable and rapid mean of diagnosis.
D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D001784:Blood Gas Analysis; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000175:diagnosis; D008297:Male; D010951:Plasma Exchange; D012129:Respiratory Function Tests
Actualité du syndrome de Goodpasture.