Abstract Details

Title Use of Commercial Claims Data to Estimate Hereditary Transthyretin-Mediated Amyloidosis Prevalence and Incidence in the US

Topic General Neurology

Presentation(s) Neuroepidemiology Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 012

Background Current prevalence and incidence estimates of transthyretin-mediated (ATTR) amyloidosis, including the hereditary mutation (ATTRv), in the US remain uncertain. Prevalence of ATTRv amyloidosis has been estimated to be ≤6,400 cases among the US population; however, these estimates are based on small samples and older data.

Objective To investigate the feasibility of estimating hereditary transthyretin-mediated (ATTRv) amyloidosis prevalence and incidence, using a large US insurance claims database.

Design/Methods We first identified patients ≥18 years believed to be diagnosed with ATTR amyloidosis in IBM^® MarketScan^® Commercial and Medicare Supplemental database (patients ≥65 years underrepresented). Diagnosis required ≥1 inpatient or ≥2 outpatient claims with an ICD-10-CM code for hereditary (E85.1,E85.2) or wild-type (E85.82) form in 2018 or another amyloidosis form in 2018 plus the following between 2014-2018: ≥1 claim for congestive heart failure, cardiomyopathy, or neuropathy; and no chemotherapy, stem cell transplant, or light-chain amyloidosis claims. Patients with ATTR identified above and lacking code E85.82 were then identified as ATTRv. Patients with dementia were excluded. Prevalence of ATTRv was number of cases divided by all members enrolled on 6/30/2018; incidence of ATTRv was number of incident cases (no amyloidosis claim in 2017) divided by total at-risk patient-years from 1/1/2018 to diagnosis (cases) or enrollment end (non-incident members) in 2018.

Results We estimated prevalence of ATTRv amyloidosis in 2018 to be 8.22 per million; highest among patients ≥65 years (42.18) and among males (9.95) vs. females (6.64), all per million. ATTRv incidence in 2018 was 4.46 per million person-years (PMPY); highest among those ≥65 years (24.41) and among males (5.67) vs. females (3.37), all PMPY.

Conclusions This study developed a commercial claims-based algorithm to estimate prevalence and incidence of ATTRv in the US. This algorithm may improve understanding of ATTRv epidemiology.

Authors/Disclosures
Duncan Brown (Akcea Therapeutics, Inc) PRESENTER	Duncan Brown has received personal compensation for serving as an employee of Akcea Therapeutics, Inc. Duncan Brown has received stock or an ownership interest from Akcea Therapeutics, Inc.
Montserrat Vera Llonch	Montserrat Vera Llonch has received personal compensation for serving as an employee of Akcea. Montserrat Vera Llonch has received stock or an ownership interest from Akcea.
	No disclosure on file
	No disclosure on file
Eunice Chang	No disclosure on file
Marian Tarbox (Partnership for Health Analytic Research, LLC)	No disclosure on file
	No disclosure on file
	No disclosure on file