COURSE AND PROGNOSIS OF ANTIBASEMENT MEMBRANE ANTIBODY (ANTI-BM-AB)-MEDIATED DISEASE - REPORT OF 35 CASES

Anti-basement membrane antibody (anti-BM Ab) mediated disease is repor ted to be a rare disorder frequently leading to severe deterioration o f renal function. It was our purpose to work out parameters necessary to predict the outcome reliably and to examine, who will benefit most from therapy. Data from 35 patients were evaluated retrospectively. Di agnosis was based on the detection of linear IgG staining (n = 28) alo ng the glomerular basement membrane (GBM) in renal biopsies and/or on the demonstration of anti-BM Ab both by ELISA and immunoblotting (n = 35). Patients were followed up for at least 6 months. Several variable s were analysed as to whether they are appropriate prognostic factors. Twenty patients (57%) presented with Goodpasture's syndrome, 13 (37%) had anti-GBM glomerulonephritis alone, whereas two patients suffered solely from pulmonary haemosiderosis. Frequent initial symptoms were h aemoptysis (n = 18), haematuria (n = 26), proteinuria (n = 26) and ele vated serum creatinine (n = 27). Among all, 10 patients improved, havi ng stable renal function. Twenty-one patients developed end-stage rena l failure and four died. Parameters indicating a poor prognosis were a serum(s)-creatinine greater than 600 mumol/l and crescent formation i n more than 50% of the glomeruli on renal biopsy. By combining these t wo parameters the outcome could be reliably predicted. The initial ant ibody titre and cigarette smoking were without predictive value. In co nclusion, the earlier therapy starts, the better will be the result. P atients presenting early with a serum creatinine < 200 mumol/l and wit hout severe glomerular alterations gained the most benefit from therap y, indicating that outcome may be improved by early diagnosis.