Expert opinion on monitoring symptomatic hereditary transthyretin-mediated amyloidosis and assessment of disease progression

Table 1 Symptomatology of ATTRv amyloidosis

Impairment	Site of amyloid deposition	Associated symptoms or conditions
Bilateral sensorimotor polyneuropathy [1, 3]	Somatic nerve fibers	Neuropathic pain or numbness in hands and feet
		Walking difficulties, balance disorders
		Loss of grip strength
Autonomic dysfunction [14, 15]	Autonomic nerve fibers	Sexual dysfunction
		Disturbances in GI motility
		Urinary disorders
		Sweating abnormalities detected by clinical tests
		Eye dryness
Infiltrative cardiomyopathy [16,17,18,19,20]	Cardiac extracellular matrix	Dyspnea, peripheral edema
		Decrease of performance/6-min walk test
		Syncope
		Fatigue
		Bradyarrhythmias or tachyarrhythmias
Cardiac dysautonomia [14, 21,22,23]	Autonomic cardiac nerves	Lack of increase in heart rate during exercise
		Orthostatic hypotension
		Syncope
Ophthalmic impairment [15, 22]	Eye	Blurred vision
		Vitreous opacities
		Glaucoma
Connective tissue manifestations [15, 24,25,26,27]	Tenosynovial tissues, ligaments, tendons	Carpal tunnel syndrome

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