source: Amyloidosis Research Consortium
year: 2016 summary/abstract:
Amyloidosis is a disorder caused by proteins that possess abnormal conformational features, leading them to aggregate and deposit in tissues in the form of amyloid fibrils. Systemic amyloidosis is a disease that can damage the heart, kidneys, liver, soft tissue, and nervous system, resulting in multi organ failure and death. There are several types of systemic amyloidosis. The most prevalent systemic amyloidosis are amyloid light chain(AL) amyloidosis, caused by the deposition of the immunoglobulin light chain, and transthyretin (ATTR) amyloidosis, caused by the accumulation of transthyretin.
The estimated incidence of AL amyloidosis is 8.9 to 14 persons per million per year in the United States. Of these, an estimated 10% to15% of cases occur in association with multiple myeloma. Clinical presentation of AL amyloidosis can vary widely and depends on the extent and number of organs affected. Symptoms at onset frequently occur in many common diseases and are relatively general (e.g., weight loss, fatigue), and consequently accurate and early diagnosis is challenging.