Lane, T; Pinney, JH; Gilbertson, JA; Hutt, DF; Rowczenio, DM; Mahmood, S; Sachchithanantham, S; ... Lachmann, HJ; + view all Lane, T; Pinney, JH; Gilbertson, JA; Hutt, DF; Rowczenio, DM; Mahmood, S; Sachchithanantham, S; Fontana, M; Youngstein, T; Quarta, CC; Wechalekar, AD; Gillmore, JD; Hawkins, PN; Lachmann, HJ; - view fewer (2017) Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre. Amyloid: The Journal of Protein Folding Disorders , 24 (3) pp. 162-166. 10.1080/13506129.2017.1342235.

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Abstract

Objective: Systemic AA amyloidosis is a serious complication of chronic inflammation; however, there are relatively few published data on its incidence. We investigated the changing epidemiology of AA amyloidosis over a 25-year period at a single national referral centre. // Methods: We conducted a retrospective study of all patients diagnosed with AA amyloidosis who had attended the centre between 1990 and 2014 inclusive. Six hundred and twenty-five patients were studied in three cohorts: C1: 1990–1997; C2: 1998–2006; C3: 2007–2014. // Results: Mean age at presentation increased from 46 in C1 to 56 in C3 (p < .0001). The proportion of South Asian patients increased from 4% in C1 to 17% in C3 (p = .0006). Comparison of underlying diseases between C1 and C3 revealed a reduction in patients with juvenile idiopathic arthritis from 25% to 2% (p < .0001), but an increase in patients with chronic infection due to intravenous recreational drug use from 1% to 13% (p < .0001), and uncharacterized inflammatory disorders from 10% to 27% (p <.0001). More patients were in end-stage renal failure at presentation in C3 (29%) than C1 (15%) (p = .0028). Median age at death was later in C3 (62 years) than C1 (54 years) (p = .0012). // Conclusion: These data suggest both falling incidence and better outcome in AA amyloidosis over a quarter of a century, reflecting advances in therapeutics and overall management of complex chronic disease in an ageing population. AA amyloidosis of uncertain aetiology presents an emerging major problem. Newer techniques such as next-generation sequencing may aid diagnosis and effective treatment, thereby improving overall survival.

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