Systemic Amyloidosis due to Monoclonal Immunoglobulins: Cardiac Involvement.

Amyloid light chain amyloidosis (AL) is the most commonly diagnosed systemic form of amyloidosis, resulting from deposition of amyloid fibrils into various organs, such as the heart. Over the past several decades, significant advances in diagnosis and treatment have reduced overall mortality. Short-term survival, however, has not improved, in large part due to cardiovascular mortality from advanced AL cardiac amyloidosis. Early clinical suspicion of cardiac involvement is critical in order to initiate appropriate treatment and referrals for successful management. This review discusses the current challenges in diagnosis as well as available treatment options for different stages of cardiac involvement.
Competing Interests: Disclosure Dr M.S. Maurer reports grant support from National Institutes of Health (R01HL139671-01), (R21AG058348), and (K24AG036778); consulting income from Pfizer, GSK, Eidos, Prothena, Akcea, and Alnylam; and Columbia University Irving Medical Center received clinical trial funding from Pfizer, Prothena, Eidos, and Alnylam. Dr A.R. Patel and Dr S.E. Saith have no financial disclosures.
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