Amyloidosis is a rare disorder that involves the deposition of misfolded protein in extracellular tissue. Disease manifestations vary depending on the affected organs. Areas most often involved include the kidneys, gastrointestinal tract, heart, nervous system, and musculoskeletal system. The most common type is AL amyloidosis, in which plasma cells produce an abundance of a monoclonal proteins that affect numerous organ systems. A second variety, AA amyloidosis, is associated with inflammatory diseases such as rheumatoid arthritis and inflammatory bowel disease. Finally, a heritable version of amyloidosis mediated by mutations in the transthyretin protein (TTR) has a predilection for deposition in peripheral nerves and cardiac tissue1.
Lohr, MD, Kristin and Kasprowicz, MD, Eric
"Case Report: Diagnosis of Dual-Biopsy Negative Severe Cardiac Amyloidosis,"
The Medicine Forum: Vol. 17
, Article 6.
Available at: http://jdc.jefferson.edu/tmf/vol17/iss1/6