Epidemiology

Amyloidosis is rare. Immunoglobulin light chain (AL) amyloidosis is the most common type.[4]

Worldwide, the crude annual incidence of AL amyloidosis is estimated at 10.44 cases per million population (PMP), ranging from 6.72 PMP in Brazil to 14.3 PMP in Japan.[5]

One US-based study reported an increase in prevalence of AL amyloidosis from 15.5 cases per million in 2007 to 40.5 cases per million in 2015.[6] The prevalence and incidence of AL amyloidosis is higher in males than in females.[6] Average age at diagnosis is reported to be between 63 and 65 years, but patients can present at any age.[6][7]

Studies assessing ethnic disparities in AL amyloidosis are sparse. Potential underdetection of cardiac amyloidosis among black Americans has been reported in one cohort study.[8] In another cohort of US patients with AL amyloidosis, self-identified ethnic minorities (including non-Hispanic black and Hispanic) accounted for 334 (14%) of 2416 patients from a single referral centre. This percentage is lower than the reported >36% representation of racial/ethnic minorities in the US general population, suggesting underdetection of AL amyloidosis among ethnic minorities.[9] Of note, the incidence of multiple myeloma and monoclonal gammopathy of undetermined significance (MGUS), both closely related to amyloidosis, is approximately two- to threefold greater in black people than in white people.[10][11][12]​ The incidence of monoclonal gammopathy disorders is lower in Asian people compared with white people.[12]

Transthyretin (TTR) amyloidosis has historically been considered a rare condition. Precise estimates of incidence and prevalence are not available, but growing evidence suggests that it is more common than previously assumed and often goes undiagnosed in patients with cardiomyopathy.​[13][14]​​​​

The incidence of secondary (AA) amyloidosis in the Western world has been falling, possibly due to advances in treatment for chronic inflammatory diseases.[15][16][17]​ Epidemiological study data indicate that, in 2008, AA amyloidosis accounted for approximately 18% of incident systemic amyloidoses in England.[18]

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