{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,4]],"date-time":"2026-11-04T15:06:15Z","timestamp":1793804775053,"version":"4.6.1"},"reference-count":43,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2025,12,30]],"date-time":"2025-12-30T00:00:00Z","timestamp":1767052800000},"content-version":"unspecified","delay-in-days":182,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"AstraZeneca Rare Disease"},{"DOI":"10.13039\/100006396","name":"Alexion Pharmaceuticals","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006396","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2025,12,15]]},"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Amyloid transthyretin (ATTR) amyloidosis is a rare, life-threatening disease frequently manifesting with cardiomyopathy (ATTR-CM), polyneuropathy (ATTR-PN) or both (ATTR-mixed). We retrospectively analysed US electronic health records and claims data to provide up-to-date estimates of ATTR amyloidosis epidemiology (overall and by phenotype).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data were extracted from the Clarivate Real-World Data repository (2016\u22122023). Given the lack of established coding for ATTR amyloidosis, we used different combinations of diagnostic codes to obtain narrow and broad estimates of incident and prevalent cases in the USA in 2022. Temporal trends (2019\u20132022) were also assessed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Using narrow definitions, the 2022 estimated incidence of ATTR amyloidosis overall, ATTR-CM, ATTR-PN and ATTR-mixed was 16.6, 12.7, 3.5 and 1.9 cases per million people, respectively; the corresponding prevalence estimates were 59.8, 41.1, 15.1 and 9.8 cases per million people. Estimates were consistently lower with the narrow (vs broad) definitions. Over time, the incidence and prevalence of ATTR amyloidosis overall increased, driven by ATTR-CM cases. No major changes were reported for the other phenotypes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This study provides comprehensive and up-to-date epidemiological data for ATTR amyloidosis in the USA. Our findings corroborate the need for appropriate differential diagnostic coding and standardised criteria.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/openhrt-2025-003781","type":"journal-article","created":{"date-parts":[[2025,12,30]],"date-time":"2025-12-30T19:15:16Z","timestamp":1767122116000},"page":"e003781","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":6,"title":["Prevalence and incidence of amyloid transthyretin amyloidosis in the USA: insights from claims databases and electronic health records"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1930-7913","authenticated-orcid":false,"given":"Pedro A","family":"Laires","sequence":"first","affiliation":[{"name":"Epidemiology, Alexion, AstraZeneca Rare Disease, Barcelona, Spain"},{"name":"Public Health Research Center, Universidade Nova de Lisboa, Lisbon, Lisbon, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaolei","family":"Li","sequence":"additional","affiliation":[{"name":"Clarivate Analytics US LLC, Philadelphia, Pennsylvania, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aishwarya M","family":"Uday","sequence":"additional","affiliation":[{"name":"Clarivate Analytics US LLC, Philadelphia, Pennsylvania, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Candida Cristina","family":"Quarta","sequence":"additional","affiliation":[{"name":"Clinical Development, Alexion, AstraZeneca Rare Disease, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Martins","family":"Silva","sequence":"additional","affiliation":[{"name":"Clinical Development, Alexion, AstraZeneca Rare Disease, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,12,30]]},"reference":[{"key":"2025123021350658000_12.2.e003781.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2015.09.075"},{"key":"2025123021350658000_12.2.e003781.2","doi-asserted-by":"crossref","DOI":"10.1186\/s12891-020-03912-z","article-title":"Musculoskeletal pathology as an early warning sign of systemic amyloidosis: a systematic review of amyloid deposition and orthopedic surgery","volume":"22","author":"Wininger","year":"2021","journal-title":"BMC Musculoskelet Disord"},{"key":"2025123021350658000_12.2.e003781.3","doi-asserted-by":"crossref","DOI":"10.1186\/s13023-023-02962-5","article-title":"A 15-year consolidated overview of data in over 6000 patients from the Transthyretin Amyloidosis Outcomes Survey (THAOS)","volume":"18","author":"Gentile","year":"2023","journal-title":"Orphanet J Rare Dis"},{"key":"2025123021350658000_12.2.e003781.4","unstructured":"Delgado DS , Pao C , Dabbous F , eds. 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