Cardiac amyloidosis--an update.

Amyloidosis is a rare, mysterious and serious disorder. The extracellular deposition of seemingly homogenous and amorphous material actually represents the expression of very diverse pathologies. All amyloid fibrils share apple green birefringence under polarized light with Congo-red staining, and are arranged in beta-pleated structure on electron microscopy. More than 21 proteins have been identified that may give rise to amyloid in vivo including light chain immunoglobulins, transthyretin, acute phase reactant protein A, fibrinogen Aα, apolipoprotein A etc. There are similarities and differences in the clinical manifestations of amyloidosis resulting from different precursor proteins. An accurate diagnosis of the type of amyloid has now become important as therapy for some forms of amyloidosis is emerging, and also the prognosis varies amongst different amyloidoses. Exciting progress is being made in our understanding of the process of amyloidogenesis, mechanisms of tissue damage due to amyloidosis, in genetics of amyloidosis and related areas 5 raising the hope of effective treatment of the disease. 6 However, a cure for all patients with amyloidosis is still afar. At a clinical level, systemic amyloidosis is a disease with protean manifestations and the diagnosis is often delayed in the era of compartmentalized care. An early diagnosis may facilitate therapy before the end organ damage is severe. Cardiac involvement is the cause of death in nearly 50% of patients with systemic amyloidosis. In this article, we highlight the selected aspects of cardiac amyloidosis from a clinical standpoint and review the recent progress in the treatment of amyloidosis.