Amyloidosis: A Clinical Overview

作者：Hazenberg Bouke P C^*

来源：Rheumatic Disease Clinics of North America, 2013, 39(2): 323-+.

DOI：10.1016/j.rdc.2013.02.012

• Systemic amyloidosis
• Amyloid fibril
• Protein misfolding
• Precursor protein
• Typing
• Diagnosis
• Treatment
• Disease monitoring

Amyloidosis is the name for protein-folding diseases characterized by extracellular deposition of a specific soluble precursor protein that aggregates in the form of insoluble fibrils. The classification of amyloidosis is based on the chemical characterization of the precursor protein. Deposition of amyloid is localized or systemic. The 4 main types of systemic amyloidosis are AL, AA, ATTR, and A beta M-2 type. A schematic approach is proposed for the clinical management of systemic amyloidosis. The importance of typing amyloid with confidence, the usefulness of imaging techniques, the principles of treatment, and the need for well-planned treatment monitoring during follow-up are discussed.

• 出版日期2013-5