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Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre

Thirusha Lane, Jennifer H. Pinney, Janet A. Gilbertson, David F. Hutt, Dorota M. Rowczenio, Shameem Mahmood, Sajitha Sachchithanantham, Marianna Fontana, Taryn Youngstein, Candida C. Quarta, Ashutosh D. Wechalekar, Julian D. Gillmore (+2 others)
2017 Amyloid: Journal of Protein Folding Disorders  

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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. 625 patients were studied in three cohorts Results Mean age at presentation
more » ... creased from 46 in C1 to 56 in C3 (p < 0.0001). The proportion of South Asian patients increased from 4% in C1 to 17% in C3 (p = 0.0006). Comparison of underlying diseases between C1 and C3 revealed a reduction in patients with JIA from 25% to 2% (p < 0.0001), but an increase in patients with chronic sepsis due to IVDU from 1% to 13% (p < 0.0001), and uncharacterised inflammatory disorders from 10% to 27% (p <0.0001). More patients were in ESRF at presentation in C3 (29%) than C1 (15%) (p = 0.0028). Median age at death was later in C3 (62 years) than C1 (54 years) (p = 0.0012). 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 aging 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.
doi:10.1080/13506129.2017.1342235 pmid:28686088 fatcat:272mzgiqvjer3kfzv24une5bem
Citation

Thirusha Lane, Jennifer H. Pinney, Janet A. Gilbertson, David F. Hutt, Dorota M. Rowczenio, Shameem Mahmood, Sajitha Sachchithanantham, Marianna Fontana, Taryn Youngstein, Candida C. Quarta, Ashutosh D. Wechalekar, Julian D. Gillmore, Philip N. Hawkins, Helen J. Lachmann. "Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre." Amyloid: Journal of Protein Folding Disorders 24.3 (2017) 162-166