[Anti-basement-membrane antibody mediated, rapidly progressive, glomerulonephritis. Diagnostic and therapeutic strategy based on a retrospective study of 14 cases].
Fourteen cases of anti-GBM antibody-induced RPGN were evaluated retrospectively in terms of renal function improvement and therapeutic risks. Nine men and 5 women (mean age: 55.3 years) were observed over a 9 year period; in three patients, hemoptysis was associated with renal disease (Goodpasture's syndrome). Most of these patients had received combinations of steroid therapy (ST), immunosuppressive drugs (IS) and plasma exchanges (PE). Age, duration of symptoms prior to diagnosis, initial renal function, therapeutic modalities and complications were assessed according to renal outcome: 9 patients (group A, "non-responders") remained on dialysis irrespective of the treatment administered; 5 patients (group B, "responders") recovered renal function. Complications, especially infections, were twice as frequent in group A. Two of the 4 recorded deaths were related to the disease or the treatment. Analysis of clinical and pathological values at the time of entry into the study for both groups indicated that oliguria/anuria, serum creatinine greater than 500 mumol/l and greater than 50% crescents, when associated, were factors predictive of poor renal outcome; in these patients, dialysis may be required except in cases of pulmonary hemorrhage. In all other patients, treatment with ST, IS and PE is recommended. Active hemoptysis necessitates pulse steroids or PE; if absent, further tests (carbon monoxide uptake, bronchoalveolar lavage, lung biopsy) are indicated before use of aggressive therapy.
Bouget
J
J
Service d'Accueil-Urgence Médicales, CHRU, Hôpital Pontchaillou, Rennes.
Le Pogamp
P
P
Perrier
G
G
Ramée
M P
MP
Rivalan
J
J
Camus
C
C
Thomas
R
R
Chevet
D
D
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D019867:Anti-Glomerular Basement Membrane Disease / Q000276:immunology; D000888:Antibodies, Anti-Idiotypic / Q000276:immunology; D001485:Basement Membrane / Q000276:immunology*; D005260:Female; D005921:Glomerulonephritis / Q000150:complications / Q000276:immunology* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007678:Kidney Glomerulus / Q000276:immunology; D008171:Lung Diseases / Q000150:complications; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011379:Prognosis; D012189:Retrospective Studies
D000305:Adrenal Cortex Hormones; D000888:Antibodies, Anti-Idiotypic; D007166:Immunosuppressive Agents
Glomérulonéphrites rapidement progressives à anticorps anti-membrane basale glomérulaire. Stratégie diagnostique et thérapeutique à partir d'une étude rétrospective de 14 cas.