**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Pedro A. Laires, Alexion - AstraZeneca Rare Disease, Barcelona, Spain

**Main Body of the Abstract**
Objectives: This study aimed to describe the epidemiology of light chain (AL) amyloidosis in US.
Methods: A retrospective analysis (January 01, 2017 - December 31, 2021) was conducted using de-identified patient records from Optum's Electronic Health Records (EHR). Adults were identified based on ≥2 AL diagnosis codes (ICD-10-CM E85.81) or positive disease mentions (including clinical features indicative of AL) found in physician notes (≥30 days apart).
Results: 1471 AL patients were included in the study population. Mean age of prevalent patients was 67.2 years, with 54.5% male. The diagnosed prevalence of AL substantially increased from 22.7 to 69.1 cases per million between 2017 and 2021, with an annual growth rate of 32.1%. In 2021, the annual incidence of AL was estimated to be 16.7 patients per million in the general population, with an annual growth rate of 7.7% since 2019, at which time incidence was 14.4 patients per million. Distribution of patients by European Modification of Mayo stage at diagnosis among those with available cardiac biomarker information (n=613, 42% of total sample) was: Stage I, 27%; Stage II, 42%; Stage IIIa, 16%; and Stage IIIb, 15%.
Conclusions: This study provides a contemporary report of the epidemiology of AL using a large-scale US EHR database. The observed rise in AL prevalence and incidence is likely due to a combination of increased disease awareness and improvements in detection and management, reinforcing the importance of continuous monitoring of the epidemiology of AL in the US.



**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Pedro A. Laires, _Alexion - AstraZeneca Rare Disease, Barcelona, Spain_

**Main Body of the Abstract**
**Objectives:** This study aimed to describe the epidemiology of light chain (AL) amyloidosis in US.
**Methods:** A retrospective analysis (January 01, 2017 - December 31, 2021) was conducted using de-identified patient records from Optum's Electronic Health Records (EHR). Adults were identified based on ≥2 AL diagnosis codes (ICD-10-CM E85.81) or positive disease mentions (including clinical features indicative of AL) found in physician notes (≥30 days apart).
**Results**: 1471 AL patients were included in the study population. Mean age of prevalent patients was 67.2 years, with 54.5% male. The diagnosed prevalence of AL substantially increased from 22.7 to 69.1 cases per million between 2017 and 2021, with an annual growth rate of 32.1%. In 2021, the annual incidence of AL was estimated to be 16.7 patients per million in the general population, with an annual growth rate of 7.7% since 2019, at which time incidence was 14.4 patients per million. Distribution of patients by European Modification of Mayo stage at diagnosis among those with available cardiac biomarker information (n=613, 42% of total sample) was: Stage I, 27%; Stage II, 42%; Stage IIIa, 16%; and Stage IIIb, 15%.
**Conclusions**: This study provides a contemporary report of the epidemiology of AL using a large-scale US EHR database. The observed rise in AL prevalence and incidence is likely due to a combination of increased disease awareness and improvements in detection and management, reinforcing the importance of continuous monitoring of the epidemiology of AL in the US.



Prevalence, Incidence, And Characterization Of Light Chain Amyloidosis In The Usa: A Real-World Analysis Utilizing Electronic Health Records (EHR) PA54 (#65)

We invite you to attend the XIX International Symposium on Amyloidosis to be held in Rochester, MN.

This symposium is organized as a hybrid meeting (with the focus on in-person) held from Sunday, May 26 through Thursday, May 30, 2024.

Together with the ISA Board and members of Mayo Clinic, a very attractive scientific hybrid program is being planned. The program will cover a variety of types of systemic amyloidosis: ATTR (hereditary and wild-type), AL (light-chain), AA and less frequent forms. There will be key lectures, specific symposia including those company sponsored, oral presentations and poster sessions of accepted abstracts.

COURSE DESCRIPTION
This program will provide a comprehensive review of state-of-the-art education in systemic amyloidosis: basic science, pathophysiology, diagnosis, current and emerging treatments. Scientific presentations will include competitive oral and poster abstracts. The rapid evolution of scientific discovery and expanding treatment options has created tremendous interest in the field and this meeting will fill and important gap in educating scientists and clinicians.