Volume 111, Issue 5 pp. 697-705
ORIGINAL ARTICLE

Epidemiology and clinical outcomes of light-chain amyloidosis in Sweden: A nationwide population-based study

Ulf-Henrik Mellqvist

Corresponding Author

Ulf-Henrik Mellqvist

Section of Hematology and Coagulation, Department of Medicine, Sahlgrenska University Hospital, Gothenburg, Sweden

Correspondence

Ulf-Henrik Mellqvist, South Elvsborg Hospital, Sahlgrenska University Hospital, Blå stråket 5, 413 45 Göteborg, Sweden.

Email: [email protected]

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Qian Cai

Qian Cai

Janssen Global Services, Titusville, New Jersey, USA

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Laura L. Hester

Laura L. Hester

Janssen Research & Development, Horsham, Pennsylvania, USA

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Michael Grövdal

Michael Grövdal

Janssen-Cilag, Solna, Sweden

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Jakob Börsum

Jakob Börsum

SDS Life Science, Stockholm, Sweden

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Iffat Rahman

Iffat Rahman

SDS Life Science, Stockholm, Sweden

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Eric M. Ammann

Eric M. Ammann

Janssen Global Services, Raritan, New Jersey, USA

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Markus Hansson

Markus Hansson

Department of Hematology, Sahlgrenska Academy, Göteborg University and Sahlgrenska University Hospital, Gothenburg, Sweden

Department of Hematology, Skåne University Hospital, Lund, Sweden

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First published: 02 August 2023

Michael Grövdal was in Janssen-Cilag at the time this research was performed.

The data in this manuscript were presented in part at the 64th American Society of Hematology Annual Meeting.

Abstract

Objectives

This study evaluated data from six Swedish national registries to fill current evidence gaps on the epidemiology, clinical burden, and overall survival (OS) associated with light-chain (AL) amyloidosis.

Methods

Patients newly diagnosed with AL amyloidosis were identified using six linked Swedish nationwide population-based registers. For each case, individuals from the general population were selected and matched with a maximum ratio of 1:5 based on age, sex, calendar year, and county.

Results

846 patients newly diagnosed with AL amyloidosis and 4227 demographically matched individuals were identified. From 2011 to 2019, annual AL amyloidosis incidence increased from 10.5 to 15.1 cases per million. At baseline, patients with AL amyloidosis had a significantly higher disease burden including higher rates of cardiac and renal failure relative to the comparison group. Among patients with AL amyloidosis, 21.5% had incident heart failure and 17.1% had incident renal failure after initial diagnosis. Median OS for patients with AL amyloidosis was 56 months versus not reached in the matched general population comparison group.

Conclusion

The incidence of newly diagnosed AL amyloidosis in Sweden increased over time with AL amyloidosis being associated with a higher risk of cardiac/renal failure and all-cause mortality compared with the general population.

CONFLICT OF INTEREST STATEMENT

Ulf-Henrik Mellqvist has received honoraria from Janssen and Celgene; Qian Cai is a current employee of and equity holder in Janssen; Laura L. Hester is a current employee of and equity holder in Janssen; Michael Grövdal was employed by Janssen at the time of the study; Jakob Börsum is employed by SDS Life Science, Sweden; Iffat Rahman is employed by SDS Life Science, Sweden; Eric M. Ammann is a current employee of and equity holder in Janssen; Markus Hansson has received lecture honoraria from Janssen and Celgene.

DATA AVAILABILITY STATEMENT

The data sharing policy of Janssen Pharmaceutical Companies of Johnson & Johnson is available at https://www.janssen.com/clinical-trials/transparency. As noted on this site, requests for access to the study data can be submitted through the Yale Open Data Access (YODA) Project site at http://yoda.yale.edu.

The full text of this article hosted at iucr.org is unavailable due to technical difficulties.