Fast Five Quiz: Treatment of Hereditary (Familial) Amyloid Transthyretin-Related (hATTR) Amyloidosis

Jefferson R. Roberts, MD; Victoria M.F. Mank, MD

Disclosures

May 24, 2021

Analgesic agents can be administered to help ameliorate neuropathic pain experienced by patients with hATTR amyloidosis. Certain calcium channel blockers are contraindicated in hATTR amyloidosis because these agents accumulate within the amyloid deposits, thereby increasing the risk for cardiac complications. The use of diuretic agents for heart failure in patients with amyloidosis is complicated because these medications reduce preload (filling pressure). Patients often require higher filling pressure to distend a stiffened heart (related to the amyloid protein buildup within cardiac tissue). Beta-blockers, angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers should be avoided due to being poorly tolerated in cardiac amyloidosis. Nonsteroidal anti-inflammatory therapy has been shown to improve quality of life scores and reduce neurologic impairment compared with placebo. In a randomized, placebo-controlled trial in patients with stage I-II transthyretin-related familial amyloid polyneuropathy (TTR-FAP), the nonsteroidal anti-inflammatory drug (NSAID) diflunisal (approved for the treatment of arthritis) improved quality-of-life scores and reduced progression of neurologic impairment compared with placebo, but its use in the setting of hATTR amyloidosis remains off-label.

Learn more about clinical manifestations in patients with hATTR amyloidosis, as well as treatment options.

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