Familial amyloidosis epidemiology and demographics

The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide. The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries. In amyloidosis, the mean age of presentation is 55 – 60 years. Men are more commonly affected by amyloidosis than women.

• The incidence of amyloidosis is approximately 1.2 per 100,000 individuals per year worldwide.^[1]

• The mortality rate of systemic amyloidosis is approximately 100 per 100,000 deaths in developed countries.^[2]

• In familial amyloidosis, the mean age of presentation can vary based on the type of protein involved:^{[3][4][5][6][7][8][9][10]}
  • Transthyretin (TTR): After 50 years of age
    • The age of onset in TTR Val30Met variant of the disease in northern Portugal is in the early 30s
  • Apolipoprotein AI: Third decade and older
  • Apolipoprotein AII: Early adulthood
  • Fibrinogen Aa: Fourth to fifth decade
  • Lysozyme: Third to fourth decade
  • Gelsolin: Late adulthood
  • Cystatin C: Third to fourth decade

• Hereditary amyloidosis subtypes include a substitution of an amino acid that is detected in approximately 4% of the African American population.^[11]

• Men are more commonly affected by amyloidosis than women.^[12]

• 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.^{[13][14][15][16][17][18][19][20][21][22][23]}
• 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 majority of gelsolin related amyloidosis cases are reported in the United States, Japan, Portugal, England, Germany, Spain, France, Brazil, Sewden, Denmark, the Czech Republic, and Iran.

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