A new era of amyloidosis: the trends at a major US referral centre. (2nd October 2019)

• Record Type: Journal Article
• Authors: Staron, Andrew; Connors, Lawreen H.; Ruberg, Frederick L.; Mendelson, Lisa M.; Sanchorawala, Vaishali
• Is Part Of: Amyloid. Volume 26:Number 4(2019)
• Page Start: 192
• Page End: 196
• Publication Date: 2019-10-02
• Subjects: Amyloidosis -- referral -- ATTR -- AL -- localized amyloidosis -- tertiary centre; Amyloidosis -- Periodicals; 616.3995
• DOI: 10.1080/13506129.2019.1640672
• Languages: English
• ISSNs: 1350-6129

Abstract

Abstract: Objective: To characterize the changing spectrum of amyloidosis classes, as well as patient demographics, at a major US referral centre. Patients and methods: A retrospective analysis was conducted of all referrals to the Amyloidosis Centre at Boston University and Boston Medical Centre over the last 3 decades. Results: A total of 3987 new patients with amyloidosis were evaluated between 1990 and 2018 with the average number of new cases per year increasing 2.5-fold during this period. Systemic immunoglobulin light-chain (AL) amyloidosis decreased in proportion with each decade from 77% to 69% to 50% of new cases. Meanwhile, ATTR amyloidosis increased from 12% to 16% to 29%, predominately due to more diagnosis of ATTRwt and ATTRV122I amyloidosis. Gender and race profile differences, while changing over the observed time period, persisted among amyloidosis patients. Conclusion: Amyloid diseases are more widely recognized and classes of amyloidosis, including ATTRwt and ATTRV122I, once considered rare are now increasingly diagnosed. These data likely reflect a national trend of increased amyloidosis awareness facilitated by accessible diagnostic approaches, emerging treatments, and coordinated educational initiatives. Trial registration: ClinicalTrials.gov identifier: NCT00898235.