What is the incidence of AA (Amyloid A) amyloidosis?

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Incidence of ATTR Amyloidosis

The question asks about ATTR amyloidosis, but the provided evidence exclusively addresses AL (light chain) and AA (amyloid A) amyloidosis, with no specific incidence data for ATTR amyloidosis available in these sources.

Important Clarification

The evidence provided does not contain incidence data for ATTR (transthyretin) amyloidosis, which is a distinct entity from:

  • AL amyloidosis (light chain amyloidosis associated with plasma cell disorders) 1
  • AA amyloidosis (reactive amyloidosis secondary to chronic inflammatory conditions) 2, 3

Available Incidence Data from Evidence (Not ATTR)

AL Amyloidosis Incidence

The incidence of AL amyloidosis ranges from 3 to 13 cases per million population per year across Western countries 1:

  • United States: 8-14 cases per million per year 1
  • United Kingdom: 3 cases per million per year 1
  • Sweden: 3.2 cases per million per year 1, 4
  • Netherlands: 6 cases per million per year 1
  • Italy: 13.29 cases per million per year 1
  • Regional estimates (Minnesota, Florence, Limousine): approximately 12 cases per million per year 1

AA Amyloidosis Incidence

AA amyloidosis has an estimated incidence of approximately 1-2 cases per million population per year 4, 2:

  • Sweden: approximately 2.0 cases per million per year (when accounting for rheumatoid arthritis-related deaths) 4
  • France: estimated 500-700 total cases in the entire population 3

ATTR Amyloidosis Context (Limited Data Available)

The only reference to ATTR in the evidence indicates:

  • Variant (hereditary) ATTR: estimated incidence of 0.3 cases per million per year 5
  • Wild-type ATTR: prevalence estimate of 155-191 cases per million persons (not incidence) 5

Critical Caveat

ATTR amyloidosis is fundamentally different from AL and AA amyloidosis in pathophysiology, clinical presentation, and epidemiology 6, 7. ATTR more commonly affects the heart, peripheral nerves, and musculoskeletal system, whereas AL amyloidosis predominantly affects kidneys and heart 6. The incidence data for AL and AA amyloidosis cannot be extrapolated to estimate ATTR incidence.

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Guideline

Localized vs Systemic Amyloidosis

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Guideline

Pulmonary Amyloidosis Types and Clinical Implications

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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