ANTI-GBM DISEASE - PREDICTIVE VALUE OF CLINICAL, HISTOLOGICAL AND SEROLOGICAL DATA

Twenty-nine patients with anti-glomerular basement membrane (GBM) dise ase, defined by circulating anti-GBM antibodies and/or linear deposits of immunoglobulin G along GBM, were studied retrospectively to identi fy prognostic indicators of renal outcome and pulmonary involvement. P atients consisted of 18 males and 11 females aged 6 to 76 years (mean 35.2 +/- 20.2). Goodpasture syndrome developed in 14, disease was conf ined to renal manifestations in 14 and isolated pulmonary involvement was present in a single case. Pulmonary disease was significantly asso ciated with current smoking (p < 0.01). Among the 29 patients, end-sta ge renal failure requiring dialysis was initially observed in 16 (55%) . Ten of them had anti-GBM glomerulonephritis (GN) and 4 presented wit h Goodpasture syndrome; the two other patients died very soon after th e beginning of the disease. The 17 patients with an unfavourable renal evolution (group 1) and the 12 patients with favourable evolution or chronic renal failure (group 2) were compared. No significant differen ce was noted between the two groups concerning the age at onset of the disease, sex, cigarette smoking or pulmonary involvement. Conversely, creatininemia over 600 mumol/l, oligoanuria, absence of normal glomer uli, a high percentage of circumferential crescents, circulating anti- GBM antibodies detected by immunofluorescence, and a high level of cir culating anti-GBM antibodies evaluated by ELISA were features which in dicate an unfavourable renal course (p < 0.01).