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Manifestations of transthyretin amyloidosis (ATTR)

RECOGNISING THE CONSTELLATION OF ATTR AMYLOIDOSIS SIGNS AND SYMPTOMS IS IMPORTANT

Early recognition of the signs and symptoms of ATTR amyloidosis can lead to earlier diagnosis and treatment which may improve patient outcomes in this progressive and potentially fatal disease1-4

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Recognising the signs and symptoms of ATTR amyloidosis could help accelerate earlier identification of the disease1

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Click or tap categories to view additional red flags.

Tap categories to view additional red flags.

SENSORIMOTOR NEUROPATHY CARDIAC MUSCULOSKELETAL AUTONOMIC DYSFUNCTION NEPHROPATHY

SENSORIMOTOR NEUROPATHY1

foot1

Painful neuropathy
in hands and feet

Falls

Falls

difficulty-walking1

Difficulty walking

Muscle weakness

Muscle weakness

CARDIAC1

cardiac-heart-failure-light

HF (right-sided), HFpEF, or intolerance to ACEi, ARBs, ARNi, or beta blockers

cardiac-Atrial-Fibrillation-dark

Atrial fibrillation

cardiac-Pacemaker

Bradyarrhythmias/conduction abnormalities/need for pacemaker

MUSCULOSKELETAL1,5–7

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Back pain/lumbar spinal stenosis

musculo-Popey-Syndrome

Ruptured distal biceps tendon/Popeye sign

musculo-Knee-Pain_dark

Shoulder, knee and hip pain or surgery

musculo-trigger-finger

Trigger finger

musculo-Carpal-Tunnel_dark

Bilateral carpal tunnel syndrome

AUTONOMIC DYSFUNCTION1

auto-hypotension_blood-pressure

Orthostatic hypotension

weight-loss

Unexplained weight loss

auto-stomach-issues

Diarrhoea

auto-erectile-disfunction

Erectile dysfunction

Constipation

Constipation

NEPHROPATHY4

nephro-Unbranded_icon_specimen cup

Proteinuria

nephro-Unbranded_icon_kidney

Renal failure

 

This list of signs and symptoms of ATTR is not intended to be exhaustive.

Watch this video to understand the disease course and signs/symptoms of ATTR amyloidosis

The “HIDDEN” mnemonic is a useful tool to help remember some of the commonly encountered “red flags” associated with ATTR-CM9

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HFpEF and other cardiac conditions, including atrial fibrillation, arrhythmia and atrioventricular block10

Intolerance to standard heart failure therapies (e.g. ACEi/ARBs and beta blockers)11

Discordance of QRS voltage and left ventricular wall thickness seen on echocardiography12,13

Diagnosis of carpal tunnel syndrome, distal biceps tendon rupture or lumbar spinal stenosis14,15

Echocardiography showing increased left ventricular wall thickness and/or low-flow gradient aortic stenosis and additional echocardiography parameters*12

Nervous system—autonomic nervous system dysfunction, including gastrointestinal complaints or unexplained weight loss16

Figure adapted from Sabbour H, et al. Front Cardiovasc Med. 2021.9

*Echocardiographic, speckle-strain and tissue doppler “red flags”: Heart failure normal or mid-range ejection fraction; increased wall thickness; left atrial enlargement; low stroke volume index; low-flow, low-gradient aortic stenosis (per ESC/EACTS Guidelines);17 low myocardial contraction fraction; advanced diastolic dysfunction; impaired global longitudinal strain with apical sparing; low mitral annular tissue Doppler S' (average septal and lateral annulus).8

In most patients with ATTR-CM, misdiagnosis or delayed diagnosis may lead to irretrievable loss of quality of life and disease progression of polyneuropathic and cardiac symptoms1,4

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ATTR amyloidosis is a progressive and potentially fatal disease caused by the buildup of amyloid fibrils in various tissues and organs1

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Late-stage disease impacts responsiveness to certain treatments18
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Increased burden of symptoms and disease18–20
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Decreased life expectancy1,18–20

ATTR amyloidosis progresses to mortality1,18,19

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Symptom deterioration may indicate disease progression

Worsening of symptoms can be a serious and potentially life-altering sign of disease progression1,19

cardiac-decline-signsnumbness-signs

Timely diagnosis and treatment of ATTR amyloidosis may help to improve patient outcomes4

UNDERSTAND DIAGNOSIS

Early intervention for ATTR amyloidosis may improve quality of life1–4,22–25

DISCOVER TREATMENT OPTIONS

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Get in touch

If you have any questions about ATTR amyloidosis in the United Kingdom or would like to speak to an AstraZeneca medical representative, please contact us

Get in touch

ACEi=angiotensin-converting enzyme inhibitor; ARB=angiotensin receptor blocker; ARNi=angiotensin receptor/neprilysin inhibitor; ATTR=transthretin amyloidosis; ATTR-CM=transthyretin amyloidosis with cardiomyopathy; ATTR-PN=transthyretin amyloidosis with polyneuropathy; ATTRv-PN=hereditary transthyretin amyloidosis with polyneuropathy; GI=gastrointestinal; HF=heart failure; HFpEF=heart failure with preserved ejection fraction.


References: 1. Nativi-Nicolau JN, et al. Heart Fail Rev. 2022;27(3):785–793; 2. Gonzàlez-Duarte, et al. Neuro Ther. 2020;9:135–149; 3. Keohane D, et al. Amyloid. 2017;24(1):30–36; 4. Rozenbaum MH, et al. J Comp Eff Res. 2021;10(11):927–938; 5. Gertz MA. Am J Manag Care. 2017;23(7 Suppl):S107–S112; 6. Adams D, et al. Orphanet J Rare Dis. 2021;16(1):411; 7. Tojo, K, et al. Amyloid. 2010;17(1), 32–35. 8. Gertz M, et al. BMC Fam Pract. 2020;21(1):198; 9. Sabbour H, et al. Front Cardiovasc Med. 2021;8:644508; 10. González-López E, et al. Eur Heart J. 2015;36(38):2585–2594; 11. Castaño A, et al. Eur Heart J. 2017;38(38):2879–2887; 12. Quarta CC, et al. Circulation. 2014;129:1840–1849; 13. Carroll JD, et al. Am J Cardiol. 1982;49:9–13; 14. Nakagawa M, et al. Amyloid. 2016;23:58–63; 15. Westermark P, et al. Ups J Med Sci. 2014;119:223–228; 16. Coelho T, et al. Curr Med Res Opin. 2013;29:63–76; 17. Vahanian A, et al. Eur Heart J. 2022;43(7):561–632; 18. Griffin JM, et al. JACC CardioOncol. 2021;3(4):488–505; 19. Kittleson MM, et al. J Am Coll Cardiol. 2023;81(11):1076–1126; 20. Hawkins PN, et al. Ann Med. 2015;47(8):625–638; 21. Witteles RM, et al. JACC Heart Fail. 2019;7(8):709–716; 22. Conceição I, et al. J Peripher Nerv Syst. 2016;21:5–9; 23. Coelho T, et al. J Neurol. 2019;267:1070–1079; 24. Adams D, et al. N Engl J Med. 2018;379:11–21; 25. Obici L, et al. Amyloid. 2020;27(3):1–10.

GB-62817 I April 2025

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