Unusual presentations of anti-glomerular basement membrane antibody mediated disease are associated with delayed diagnosis and poor outcome.

Anti-glomerular basement membrane antibody mediated disease (anti-GBM disease) is an uncommon cause of renal failure. Presentation is usually dramatic, with renal failure and alveolar hemorrhage leading to an early diagnosis. In contrast, we here describe 3 cases which presented with atypical features, and where diagnosis was delayed due to the presence of other factors which were initially felt sufficient to explain the clinical status. No patient recovered renal function, and one died from sepsis. We also present data on 15 patients presenting to Guy's Hospital over the past 10 years with anti-GBM disease and creatinine > 500 mumol/l. The median delay from presentation to diagnosis was 7 days, range 1-81 days. Two patients died, and only one recovered renal function. We speculate that earlier referral and renal biopsy might have improved outcome in these patients, and recommend a policy of early biopsy in all cases of acute renal failure with atypical feature, delay in recovery, or where a clearcut cause is not established.