Marked progress in AL amyloidosis survival: a 40-year longitudinal natural history study

Abstract

The recent decades have ushered in considerable advancements in the diagnosis and treatment of systemic light chain (AL) amyloidosis. As disease outcomes improve, AL amyloidosis-unrelated factors may impact mortality. In this study, we evaluated survival trends and primary causes of death among 2337 individuals with AL amyloidosis referred to the Boston University Amyloidosis Center. Outcomes were analyzed according to date of diagnosis: 1980-1989 (era 1), 1990-1999 (era 2), 2000-2009 (era 3), and 2010-2019 (era 4). Overall survival increased steadily with median values of 1.4, 2.6, 3.3, and 4.6 years for eras 1–4, respectively (P < 0.001). Six-month mortality decreased over time from 23% to 13%. Wide gaps in survival persisted amid patient subgroups; those with age at diagnosis ≥70 years had marginal improvements over time. Most deaths were attributable to disease-related factors, with cardiac failure (32%) and sudden unexpected death (23%) being the leading causes. AL amyloidosis-unrelated mortality increased across eras (from 3% to 16% of deaths) and with longer-term survival (29% of deaths occurring >10 years after diagnosis). Under changing standards of care, survival improved and early mortality declined over the last 40 years. These findings support a more optimistic outlook for patients with AL amyloidosis.

A Risk Score to Diagnose Cardiac Involvement and Provide Prognosis Information in Patients at Risk of Cardiac Light-Chain Amyloidosis

“…AL CA was proved to affect prognosis most, constituting the major cause of death ( 4 ). It is reported that, without treatment, the median survival time of AL CA patients was 6 months since the presentation of symptoms ( 5 ). However, atypical symptoms in the early stage and deficient recognition of cardiac involvement make AL CA an underdiagnosed disease ( 6 ).…”

Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment

“…The 5-year overall survival from 1980 to 1989, 1990 to 1999, 2000 to 2009, and 2010 to 2019 are 15%, 36%, 40%, and 48%, respectively. 32 The 1-year mortality rate remained high during the 30-year period (12%-30%), reflecting the failure to diagnose this rare disorder prior to irreversible end organ damage. 33 on progression-free survival in contrast to myeloma where it has no impact.…”

Arrhythmias in Chronic Kidney Disease

“…[ 17 ] Although cardiac failure is the most common cause of mortality in patients with amyloidosis, SCD remains a concern. [ 18 ] SCD can include ventricular arrhythmias but also pulseless electrical activity with electromechanical asynchrony.…”

“…17 Although cardiac failure is the most common cause of mortality in patients with amyloidosis, SCD remains a concern. 18 SCD can include ventricular arrhythmias but also pulseless electrical activity with electromechanical asynchrony. This shows the interactions between chronic kidney disease and arrhythmia and the causes and effects of each.…”