Anti-glomerular basement membrane disease in Auckland

Abstract

Background: Anti-glomerular basement membrane (GBM) antibody-mediated disease is rare and classically presents with the syndrome of glomerulonephritis and pulmonary haemorrhage.

Aim: This aim of this report was to determine the incidence, clinical features, management and outcomes of anti-GBM disease in Auckland between 1998 and 2008.

Methods: Potential patients were identified by a search for positive anti-GBM antibody serology, diagnostic renal biopsy, or in-hospital admissions using International Classification of Diseases 9 and 10 codes between 1998 and 2008. A retrospective case notes review of all potential cases was performed with data censored at 31 December 2010.

Results: Twenty-three cases were identified. The rate of anti-GBM disease was estimated at 1.79 per million person-years. There were 12 men and 11 women. The median age was 45 years, range 12-74 years. Sixteen patients were European, three were Pacific peoples, three were NZ Maori and one was Chinese. Eleven were regular smokers and eight ex-smokers, significantly higher proportions than the population (P ≤ 0.001). Smokers were significantly more likely to have respiratory disease (P= 0.03). The mean creatinine at presentation was 474 µmol/L. All patients had a renal biopsy; 20 had crescentic glomerulonephritis. One patient recovered renal function without treatment. Twenty-two were immunosuppressed with cyclophosphamide and corticosteroids. Seventeen received plasmapheresis. Eighteen were alive, eight with end-stage renal disease, two with chronic kidney disease and eight with normal renal function.

Conclusions: Anti-GBM disease is a rare condition, which is not overrepresented among indigenous people. With aggressive therapy the prognosis has improved; however, the morbidity and mortality of this condition remain significant.