Incidence and survival in non-hereditary amyloidosis in Sweden

Abstract

BackgroundAmyloidosis is a heterogeneous disease caused by deposition of amyloid fibrils in organs and thereby interfering with physiological functions. Hardly any incidence data are available and most survival data are limited to specialist clinics.MethodsAmyloidosis patients were identified from the Swedish Hospital Discharge and Outpatients Registers from years 2001 through 2008.ResultsThe incidence of non-hereditary amyloidosis in 949 patients was 8.29 per million person-years and the diagnostic age with the highest incidence was over 65 years. Secondary systemic amyloidosis showed an incidence of 1 per million and a female excess and the largest number of subsequent rheumatoid arthritis deaths; the median survival was 4 years. However, as rheumatoid arthritis deaths also occurred in other diagnostic subtypes, the incidence of secondary systemic amyloidosis was likely to be about 2.0 per million. The median survival of patients with organ-limited amyloidosis was 6 years. Most myeloma deaths occurred in patients diagnosed with unspecified or ‘other’ amyloidosis. These subtypes probably accounted for most of immunoglobulin light chain (AL) amyloidosis cases; the median survival time was 3 years.ConclusionsThe present diagnostic categorization cannot single out AL amyloidosis in the Swedish discharge data but, by extrapolation from myeloma cases, an incidence of 3.2 per million could be ascribed to AL amyloidosis. Similarly, based on rheumatoid arthritis death rates, an incidence of 2.0 could be ascribed to secondary systemic amyloidosis.

Epidemiology of AL amyloidosis: a real-world study using US claims data

“…1 In the study on the general population living in Olmsted County from 1950 to 1989, the authors found an incidence rate of 9 cases per million person-years, and deduced that ;2200 new cases of AL amyloidosis are diagnosed in the United States annually. Incidence rates found in our analyses are slightly higher than those found in England and Sweden, 11,12 likely due to the fact that these 2 studies used older data and awareness of AL amyloidosis may have increased recently.…”

“…1 Other studies, although not population-based, have been conducted in European regions. For example, in 2 separate studies, Pinney et al 11 and Hemminki et al 12 estimated AL amyloidosis incidence rates to be ;3 and 8.3 cases per million person-years in 2008 in England and Sweden, respectively. Therefore, the literature suggests an estimated incidence between 3 and 12 cases per million persons per year, and an estimated prevalence of 30 000 to 45 000 AL amyloidosis patients in the United States and the European Union.…”

“…Therefore, the literature suggests an estimated incidence between 3 and 12 cases per million persons per year, and an estimated prevalence of 30 000 to 45 000 AL amyloidosis patients in the United States and the European Union. 1,7,[11][12][13][14] Additionally, studies have shown that men have a slightly increased rate of AL amyloidosis compared with women, and the majority of patients are over the age of 65 years. 1,7,15 Given the lack of contemporary data on AL amyloidosis in the United States, this study sought to examine the prevalence (proportion of the population having the condition at a given time) and incidence (annual rate of newly identified cases) of AL amyloidosis over a 9-year period (2007-2015) using health insurance claims data.…”

Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre

“…Akse-Onal and colleagues (6) A Swedish study identifying patients from hospital discharge and outpatient registers between 2001 and 2008 estimated an incidence of AA amyloidosis of 0.2 per 100 000, based on RA death rates (7). In 2013 an epidemiological study of systemic amyloidosis in England was conducted using combined data from the United Kingdom (UK) National Amyloidosis Centre (NAC) patient database and English death certificate data from the Office of National Statistics (8).…”

Amyloidosis

“…22 In Amyloidosis a recent Swedish study, the estimated incidence of AL amyloidosis was 3.2 per million per year and AA amyloidosis 2.0 per million per year. 23 The median age for AL and AA amyloidosis is between 55 and 60 years. 24 The prognosis of untreated patients is poor, as noticed in older studies; median survival was 6 to 12 months for AL amyloidosis and 3 to 4 years for AA amyloidosis.…”