Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Jul 11;15(1):25149.
doi: 10.1038/s41598-025-09498-7.

Incidence and prevalence of light chain amyloidosis in the United States in 2019-2021 using Optum EHR data

Pedro A Laires  1   2 , Shona Fang  3 , Jennifer Evans  3 , Jeffrey Thompson  3 , Abhishek Gaur  4 , Brendon Staruk  4 , Ankita Gupta  4 , Richa Manwani  5   6
Affiliations

Incidence and prevalence of light chain amyloidosis in the United States in 2019-2021 using Optum EHR data

Pedro A Laires et al. Sci Rep. .

Abstract

Immunoglobulin light chain amyloidosis (AL amyloidosis) is among the most common forms of systemic amyloidosis. Using electronic health records (EHR) data from the United States, we aimed to estimate the incidence and prevalence of AL amyloidosis over time, to evaluate the distribution of different disease stages, and to assess patients' demographic characteristics. We conducted a retrospective cohort study using Optum EHR data from 2016 to 2022. AL amyloidosis was defined by ≥ 2 ICD-10-CM codes or positive mentions in the EHR that were ≥ 30 days apart. Incident and prevalent patients were included. Staging was assessed using cardiac biomarkers (whenever available) according to the European modification of the 2004 Mayo staging system. A total of 1976 AL amyloidosis patients were identified. In 2021, the estimated AL amyloidosis incidence was 16.7 per million person-years in adults, and the prevalence was 69.0 per million adult population. Among patients with staging available near the time of diagnosis (41.7% of incident patients), the following distribution was observed: 16.1% Stage I, 44.5% Stage II, 20.6% Stage IIIa, and 18.8% Stage IIIb. At the most recently available staging assessment, combined Stage IIIa and IIIb was more common among males (38.6% vs. 27.1%) and was more common with increasing age (25.7% for 40-49 years vs. 38.5% for 80+ years). We observed the highest prevalence of AL amyloidosis published to date. This may be due to true increased prevalence, consistent with reports of improved survival in AL amyloidosis.

Keywords: Amyloidosis; Epidemiology; Multiple myeloma.

PubMed Disclaimer

Conflict of interest statement

Declarations. Competing interests: PAL, SF, JE, JT and RM are employees of Alexion, AstraZeneca Rare Disease, and may own stock/have stock options in the company. AG, BS and AG are Optum® employees. Ethics approval and consent to participate: This study was considered not human subjects research. The data was certified as de-identified by an independent statistical expert following HIPAA statistical de-identification rules and managed according to Optum customer data use agreements.

Figures

Fig. 1
Fig. 1
Cohort attrition diagram.
Fig. 2
Fig. 2
Trends in incidence and prevalence of AL amyloidosis from 2019 to 2021.
See this image and copyright information in PMC

Similar articles

See all similar articles

References

    1. Merlini, G. et al. Systemic immunoglobulin light chain amyloidosis. Nat. Rev. Dis. Primers4(1), 38 (2018). - PubMed
    1. Gertz, M. A. & Dispenzieri, A. Systemic amyloidosis recognition, prognosis, and therapy: A systematic review. JAMA324(1), 79–89 (2020). - PubMed
    1. Sabinot, A. et al. State-of-the-art review on AL amyloidosis in Western Countries: Epidemiology, health economics, risk assessment and therapeutic management of a rare disease. Blood Rev.59, 101040 (2023). - PubMed
    1. Kyle, R. A. et al. Incidence of AL amyloidosis in Olmsted County, Minnesota, 1990 through 2015. Mayo Clin. Proc.94(3), 465–471 (2019). - PMC - PubMed
    1. Schulman, A. et al. Patient outcomes in light chain (AL) amyloidosis: The clock is ticking from symptoms to diagnosis. Eur. J. Haematol.105(4), 495–501 (2020). - PubMed

LinkOut - more resources