What is the prevalence of amyloidosis?

Prevalence of Amyloidosis

The prevalence of amyloidosis varies by type, with AL amyloidosis having a prevalence of approximately 50-65 cases per million people in Western countries, while the overall global prevalence of amyloidosis is estimated at 51 cases per million population. 1, 2

Prevalence by Amyloidosis Type

AL Amyloidosis (Light Chain)

• Most common form of systemic amyloidosis
• Prevalence in the US: Increased from 20.1 per million people in 2007 to 50.1 per million people in 2015 1
• Prevalence in Europe: 58-65 cases per million people 1
  • France: 58 cases per million people
  • Italy: 65.38 cases per million people

ATTR Amyloidosis (Transthyretin)

• Variant ATTR: Prevalence estimated at 5.2 cases per million people 3
• Wild-type ATTR: Prevalence estimated at 155-191 cases per million people 3

AA Amyloidosis (Reactive)

• Less common in developed countries but more prevalent in some European regions and developing countries 4
• Specific prevalence figures are limited in the literature

Global Perspective

According to a 2022 global epidemiological study, approximately 74,000 AL amyloidosis cases worldwide were diagnosed during the preceding 20 years, with an estimated 20-year period prevalence of 51 cases per million population 2.

Incidence Patterns

The incidence of amyloidosis provides context for understanding prevalence:

• AL amyloidosis: Approximately 10-14 cases per million people per year in the US 1, 3
• Regional variations:
  • Minnesota (US): 12 cases per million per year
  • Florence (Italy): 6.3-11.6 cases per million per year (2018-2020)
  • Limousine (France): 12 cases per million per year
  • Uppsala (Sweden): 8 cases per million per year
  • Netherlands: 6 cases per million per year
  • UK: 3 cases per million per year 1

Demographic Patterns

• Gender distribution: AL amyloidosis affects men and women equally in Western countries (ratio 1.3:1) 5
• Age: Incidence increases with age, with most cases diagnosed in older adults 1, 5
• Multiple organ involvement: More than 69% of patients have multiple organ involvement at diagnosis 6

Clinical Implications

• Underdiagnosis is common across all forms of amyloidosis 3
• Early diagnosis is critical as 25% of patients with AL amyloidosis die within 6 months of diagnosis 3
• Median survival for AL amyloidosis is 13 months, decreasing to 4 months when heart failure develops 6
• Median survival for ATTR amyloidosis is approximately 75 months 6

Diagnostic Considerations

Amyloidosis should be considered in patients presenting with:

• Adult nondiabetic nephrotic syndrome
• Heart failure with preserved ejection fraction
• Unexplained hepatomegaly
• Peripheral neuropathy with distal sensory symptoms
• Monoclonal gammopathy with atypical clinical features 3

The prevalence data underscores the importance of increased awareness among clinicians to facilitate earlier diagnosis and improve patient outcomes, as effective therapies now exist for various forms of amyloidosis that can significantly impact morbidity and mortality when initiated before severe end-organ damage occurs.