Amyloidosis

Abstract

Amyloidosis is a group of disorders characterized by over expression and extracellular deposition of amyloid with resultant organ dysfunction. The disease produces significant morbidity and mortality and its prevalence is increasing. Amyloidosis becomes clinically significant when organ function is impaired either due to loss of normal cell structure or mass effect from accumulating amyloid. The current classification of amyloidosis is based on the different types of fibrillar proteins which define organ involvement and disease manifestations. The heart is the most commonly involved organ in the chest, and progressive systolic dysfunction in a patient with ventricular hypertrophy is common. Pulmonary involvement is relatively rare and is more common in the localized form of the disease. There are systemic and localized forms of the disease, with systemic disease being more common. High resolution computed tomography (HRCT) is the modality of choice to evaluate patients with suspected tracheopulmonary amyloidosis. Cardiac MRI is the imaging modality of choice for evaluating patients with suspected amyloid cardiomyopathy.