S FERNANDO1, K CHOW1, S DAYAN1, K OOI2, K POLKINGHORNE1
1Monash Health, Clayton VIC, AUS; 2West Gippsland Health VIC, AUS
Background: Anti-GBM disease is an autoimmune condition where IgG antibodies to collagen subtypes results in devastating renal failure and concurrent pulmonary haemorrhage. It has an incidence of 1 case per 1 million population per year.
Case report: This is a case of an IgA mediated anti-GBM disease in a 66 year old female with acute on chronic renal failure who was referred for renal biopsy and further investigation. She had a serum Creatinine of 360 (from baseline 270) with 3.2g proteinuria. Subsequent renal biopsy demonstrated extensive global glomerulosclerosis with cellular crescents and marked interstitial fibrosis and tubular atrophy. Immuonstaining showed intense linear deposition of IgA along the glomerular basement membrane. The patient was commenced on an immunosuppressive regime of corticosteroids and was discharged with stable renal function.
Conclusions: IgA-mediated anti-GBM disease is very rare, with only a few cases reported worldwide. If identified early, it can be treated with immunosuppressive therapy. Although not showing any extra-renal manifestations, the extent of nephrosclerosis would imply progression to end-stage renal failure in the future.