A new era of amyloidosis: the trends at a major US referral centre

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.

Keywords:

• Amyloidosis
• referral
• ATTR
• AL
• localized amyloidosis
• tertiary centre

Acknowledgements

Authors thank the members of the multidisciplinary team for the care of the patients and acknowledge the contributions of many staff members. Authors appreciate the vision of the founding director Dr. Martha Skinner in maintaining the database.

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

The Amyloidosis Center database and repository is supported by Amyloid Research Fund of Boston University School of Medicine.