[A case of anti-basement membrane (BM) mediated disease presenting renal and pulmonary symptoms by divergent timing].
A case of 49-year-old man with anti-GBM antibody and who manifested pulmonary and renal symptoms at divergent times. Thirty-six years previously, renal disease with unneglectable degree of proteinuria was noticed. One month before admission, he was found by chance to have elevated serum creatine (Scr); 3.4 mg/dl. At admission, his Scr was 13.7 mg/dl and Hb 12.7 g/dl, TP 5.2 g/dl with 3+ proteinuria and no glucosuria. He was a heavy smoker and remained so while admitted. Renal biopsy presented fibrocellular crescents in 100% of glomeruli with striking tubulointerstitial involvement. Immunofluorescence showed linear IgG deposition along the glomerular capillary wall. Hemodialysis was instituted, and after 13 hospital days, anti-GBM antibody at admission was high at 128 U, with negative PANCA. Plasmapheresis was also performed, but on the next day pulmonary hemorrhage occurred with a concomitant rise of anti-GBM to 250 U. Thus, steroid pulse therapy was conducted in combination with plasmapheresis. Pulmonary hemorrhage subsided along with lowering of anti-GBM (48 U), but renal failure persisted. The patient died of septicemia. Based on the clinical course of the case, the term "anti-BM mediated disease" may more properly delineate the entity of the disease rather than the classical eponym "Goodpasture's disease" which requires coexistence of pulmo- and renal manifestations for definition.
Usutani
S
S
Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
Nosaka
H
H
Shirai
M
M
Akimoto
H
H
Kojima
K
K
Shimomura
M
M
Kakuta
S
S
Nagase
M
M
Sado
Y
Y
D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis* / Q000628:therapy; D001323:Autoantibodies / Q000032:analysis*; D001485:Basement Membrane / Q000276:immunology*; D015415:Biomarkers / Q000032:analysis; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D008171:Lung Diseases / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D011239:Prednisolone / Q000008:administration & dosage; D006435:Renal Dialysis; D051437:Renal Insufficiency / Q000209:etiology
D001323:Autoantibodies; D015415:Biomarkers; D011239:Prednisolone