[Goodpasture disease].
We report one case of acute renal failure with oliguria, microscopic haematuria and normocytic anemia in a 86-year old Swedish woman. A full investigation led to the diagnosis of Goodpasture disease, an isolated form of Goodpasture syndrome. Goodpasture disease is and autoimmune disorder characterized by the development of autoantibodies to the NC1 domain of the alpha3 chain of type IV collagen, found mainly in glomerular basement membranes (GBM). When the disease affects both the lung and the kidney, it is called Goodpasture syndrome but the pulmonary or renal involvement can be isolated or separated in years. Its pathogenesis is not well known. It occurs essentially in Caucasian subjects, preferentially from Nordic and Anglo-Saxon countries (higher prevalence of HLA DR B1-15 and B1-4 group). Are also mentioned, the exposure to hydrocarbons, rustproof, insecticides and greasy solvents. The annual incidence of Goodpasture syndrome is rare and has been estimated in Europe to be about 0.5 to 1 case per million inhabitants. The isolated renal form represents about 1/3 of the cases. The clinical presentation is characterized by rapidly progressive renal failure with oliguria or anuria and in case of lung involvement, pulmonary hemorrhage responsible of hemoptysis, sometimes massive. Renal biopsy and immunofluorescence analysis play a key role in the diagnosis. The presence of both linear deposits of IgG along the glomerular basement membrane (GBM) and circulating anti-GBM antibodies is of paramount importance. The treatment, which depends on the degree of renal involvement, is based on the association of corticosteroids, cyclophosphamide and plasma exchanges.
Fomegné
G
G
Service de Néphrologie, Hôpital Erasme.
Dratwa
M
M
Wens
R
R
Mesquita
M
M
Van der Straaten
M
M
Vanden Haute
K
K
Fosso
C
C
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000369:Aged, 80 and over; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D001323:Autoantibodies / Q000097:blood; D001485:Basement Membrane / Q000276:immunology; D001706:Biopsy; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007678:Kidney Glomerulus / Q000276:immunology; D008171:Lung Diseases / Q000209:etiology; D010951:Plasma Exchange
D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D007074:Immunoglobulin G; D003520:Cyclophosphamide