Amyloidosis Typing by Symptoms

Several amyloidoses are associated with aging, including systemic (ATTRwt, AApoAIV) and localized forms (atrial and seminal vesicle amyloid) [1]
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Notes: CM = cardiomyopathy; PN = peripheral neuropathy
  • Clinical manifestations are heterogeneous and may be influenced by genetic and environmental factors
  • Although certain phenotypes may typically be associated with certain amyloid types, considerable clinical overlap exists and, hence, the classification of amyloidoses on clinical grounds alone is not recommended
  • In clinical practice, it is critical to distinguish between treatable versus non-treatable amyloidosis
  • Moreover, amyloidoses with a genetic component must be distinguished from the sporadic types and systemic amyloidoses must be distinguished from the localized forms
  • Among the systemic amyloidoses, AL continues to be the most common amyloid diagnosis in the developed world; other clinically significant types include AA, ALECT2, and ATTR—which is now emerging as an underdiagnosed type in both the hereditary and wild-type setting [1]
  1. Picken, MM. The Pathology of Amyloidosis in Classification: A Review. Acta Haematol. 2020;143:322-334. doi: 10.1159/000506696.

ATTRv Amyloidosis Symptoms

Depending on the variant, ATTRv amyloidosis symptoms range from predominantly cardiomyopathic (ATTRv-CM) to predominantly polyneuropathic (ATTRv-PN), with some variants having both [1-4]
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  • Atrioventricular block, a type of heart block that occurs when the electrical signals traveling from the upper chambers of the heart (atria) to the lower chambers (ventricles) are partially or completely interrupted or delayed​
  • Bundle branch block, a condition in which electrical impulses responsible for stimulating the heartbeat are delayed or blocked​
  • Interventricular septum, the thick wall that separates the 2 lower chambers (ventricles) of the heart​
  • Nephropathy, kidney disease​
  • Neurogenic bladder, urinary conditions in which individuals lack bladder control due to a brain, spinal cord, or nerve problem
ALXN: Acoramidis ​for the Treatment of ATTR-CM: Training Deck. November 22, 2021. 34_Acoradmidis Training Deck_22Nov21 FINAL. Citing:
  1. Cuddy SAM, Faulk RH. Can J Cardiol. 2020;36:396-407.
  2. Ando Y, et al. Orphanet J Rare Dis. 2013;8:31.
  3. Maurer MS, et al. Circ Heart Fail. 2019;12:e006075.
  4. Ruberg FL, et al. J Am Coll Cardiol. 2019;73:2872-2891.
  5. Sekijima Y, et al. Orphanet J Rare Dis. 2018;13:6.
  6. Muchtar E, et al. J Intern Med. 2021;289:268-292.