Section of Hematology and Coagulation, Department of Medicine, Sahlgrenska University Hospital, Gothenburg, Sweden.
Janssen Global Services, Titusville, New Jersey, USA.
Eur J Haematol. 2023 Nov;111(5):697-705. doi: 10.1111/ejh.14063. Epub 2023 Aug 2.
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.
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.
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.
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.
本研究利用来自瑞典六个全国性登记处的数据，填补轻链（AL）淀粉样变性相关流行病学、临床负担和总体生存（OS）的现有证据空白。
使用六个瑞典全国性基于人群的登记处来确定新诊断为 AL 淀粉样变性的患者。对于每例病例，从普通人群中选择个体，并根据年龄、性别、日历年份和县与最大 1:5 的比例相匹配。
确定了 846 例新诊断的 AL 淀粉样变性患者和 4227 名年龄、性别、年份和地区匹配的个体。2011 年至 2019 年，每年的 AL 淀粉样变性发病率从每百万 10.5 例增加到 15.1 例。在基线时，与对照组相比，AL 淀粉样变性患者的疾病负担明显更高，包括心力衰竭和肾功能衰竭的发生率更高。在 AL 淀粉样变性患者中，21.5%的患者在初始诊断后出现心力衰竭，17.1%的患者出现肾功能衰竭。AL 淀粉样变性患者的中位 OS 为 56 个月，而匹配的普通人群对照组未达到。
瑞典新诊断的 AL 淀粉样变性的发病率随时间增加，与普通人群相比，AL 淀粉样变性患者发生心力衰竭/肾功能衰竭和全因死亡率的风险更高。
