Goodpasture's syndrome - four case reports.
Goodpasture's syndrome (GPS) is a rare but severe immunological disease, which is characterised by rapidly progressive glomerulonephritis and intraalveolar hemorrhage (IAH) with the presence of anti-glomerular basement membrane (GBM) antibodies. We report four cases of GP's syndrome referred to the nephrology unit at Ibn Rochd UHC in Casablanca from January 1995 to December 2003. All patients had rapidly progressive glomerulonephritis (RPGN) with proteinuria and microscopic hematuria. Elevated blood pressure was noted in one case. IAH was manifested as hemoptysis in two cases, radiological signs in three cases and confirmed by broncho-alveolar lavage in all cases. Laboratory assessment revealed anemia in all cases. Renal biopsy showed extracapillary glomerulonephritis with linear deposits of IgG along the GBM. Renal failure was severe and hemodialysis was required in all cases. All patients were treated with prednisone and cyclophosphamide and none recovered renal function. Two patients died due to severe lung hemorrhage.
Salam
N
N
Nephrology Hemodialysis Department, Ibn Rochd Hospital Center, Morocco.
Rezki
H
H
Fadili
W
W
Hachim
K
K
Ramdani
B
B
D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D001706:Biopsy; D018893:Bronchoalveolar Lavage; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006469:Hemoptysis / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone