R DAVID1, E K TAI2
1Department of Renal Medicine, Royal Prince Alfred Hospital, Sydney, Australia, 2Nephrology and Internal Medicine, Wagga Wagga Base Hospital, Wagga Wagga , Australia
Background: Systemic amyloid A (AA) amyloidosis is an uncommon complication of Crohn’s disease. The natural history is of nephrotic syndrome and progressive renal failure. Chronic inflammation stimulates the production of the amyloidogenic precursor, serum amyloid A (SAA), thus treatment of amyloidosis reflects control of the underlying disease. Once renal amyloidosis is established it is notoriously difficult to treat, due to relentless proteinuria.
Case Report: A 58-year old man with a 36-year history of active stricturing Crohn’s disease was admitted to a rural referral centre with a flare. Nephrotic syndrome was present, with critical hypoalbuminaemia <6g/L, and urine albumin creatinine ratio of 1006mg/mmol cr. A history of renal AA amyloidosis was present, diagnosed on biopsy in 2004; the patient had been lost to follow up. Proteinuria progressed to 54g per day. He was persistently hypotensive, requiring twice daily albumin infusions. Hypogammaglobulinaemia, bacteraemia and psoas abscess complicated the admission. Renal function deteriorated and he progressed to end stage kidney disease (ESKD). He was unable to be safely dialysed due to haemodynamic instability. To negate the catastrophic proteinuria, chemical nephrectomy was attempted with indomethacin and cyclosporin, but adverse effects ensued. The decision was made to perform bilateral nephrectomies. Anephric, the patient tolerated intermittent haemodialysis, and was discharged from hospital, with a future goal of renal transplantation.
Conclusions: Historically renal amyloidosis has a grave prognosis, with a majority progressing to ESKD. Recurrent sepsis and hypotension reduce survival across dialysis and transplant populations. Here we present bilateral nephrectomies as a life-saving strategy, with a hope to bridge to transplantation. Control of the underlying disease is paramount; advances in biologic agents for inflammatory conditions should portend a brighter outlook for patients.
Nephrology advanced trainee – first year