Primary amyloidosis epidemiology and demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

There are approximately 4000 new cases of AL amyloidosis annually in the United States, though actual incidence may be somewhat higher as a result of under-diagnosis. While the incidence is thought to be equal in males and females, about 60% of patients referred to amyloid centers are male. AL amyloidosis has been reported in individuals as young as 20 years of age but is typically diagnosed at about age 50-65.

• The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide.^[1]
• The incidence of AL amyloidosis in USA ranged from 1 per 100,000 to 1.4 per 100,000 from 2007 to 2015.^[2]
• Transthyretin-related hereditary amyloidosis is endemic in Portuguese locations Póvoa de Varzim and Vila do Conde (Caxinas), with more than 1000 affected people, coming from about 500 families, where 70% of the people develop the illness. In northern Sweden, more specifically Piteå, Skellefteå and Umeå, 1.5% of the population has the mutated gene. There are many other populations in the world who exhibit the illness after having developed it independently.

• The prevalence of AL in USA amyloidosis increased significantly between 2007 and 2015, from 1.6 per 100,000 in 2007 to 4.0 per 100,000 in 2015.^[3]

• The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries.^[4]

• In amyloidosis, the mean age of presentation is 55-60 years.^[5]

• Hereditary amyloidosis subtypes include a substitution of an amino acid that is detected in approximately 4% of the black population.^[6]

• Men are more commonly affected by amyloidosis than women.^[7]

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