Amyloid diseases of the heart: current and future therapies

Amyloid diseases in man are caused by as many as 23 different pre-cursor proteins already described. Cardiologists predominantly encounter three main types of amyloidosis that affect the heart: light chain (AL) amyloidosis, senile systemic amyloidosis (SSA) and hereditary amyloidosis, most commonly...

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Bibliographic Details
Published in:QJM : An International Journal of Medicine 2012-07, Vol.105 (7), p.617-631
Main Authors: DUBREY, S. W, COMENZO, R. L
Format: Article
Language:English
Subjects:
Amyloidosis - diagnosis
Amyloidosis - etiology
Amyloidosis - therapy
Biological and medical sciences
Biomarkers - metabolism
Cardiomyopathies - diagnosis
Cardiomyopathies - etiology
Cardiomyopathies - therapy
Disease Progression
General aspects
Heart Transplantation
Humans
Medical sciences
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Summary:Amyloid diseases in man are caused by as many as 23 different pre-cursor proteins already described. Cardiologists predominantly encounter three main types of amyloidosis that affect the heart: light chain (AL) amyloidosis, senile systemic amyloidosis (SSA) and hereditary amyloidosis, most commonly caused by a mutant form of transthyretin. In the third world, secondary amyloid (AA) is more prevalent, due to chronic infections and inadequately treated inflammatory conditions. Much less common, are the non-transthyretin variants, including mutations of fibrinogen, the apolipoproteins apoA1 and apoA2 and gelsolin. These rarer types do not usually cause significant cardiac compromise. Occurring worldwide, later in life and of less clinical significance, isolated atrial amyloid (IAA) also involves the heart. Heart involvement by amyloid often has devastating consequences. Clinical outcome depends on amyloid type, the extent of systemic involvement and the treatment options available. An exact determination of amyloid type is critical to appropriate therapy. In this review we describe the different approaches required to treat this spectrum of amyloid cardiomyopathies.
ISSN:1460-2725
1460-2393
DOI:10.1093/qjmed/hcr259