Summary of common systemic amyloid types
| Amyloid type . | Precursor protein . | Cause . | Organ pattern . | Primary treatment* . |
|---|---|---|---|---|
| AL | Immunoglobulin light chain | Secondary to a clonal plasma cell or lymphoproliferative disorders | Heart Kidneys Liver PNS ANS Soft tissue | Chemotherapy, autologous stem cell transplantation to control underlying clonal disorder |
| ATTRwt | Wild-type transthyretin | Aging | Heart Soft tissue | Tafamidis |
| ATTRv | Mutated transthyretin | Hereditary | Heart PNS ANS GI tract | Patisiran, inotersen, vutrisiran for neuropathy Tafamidis for cardiomyopathy |
| AA | Serum amyloid A protein | Chronic inflammation | Kidneys Liver Heart ANS | Suppress inflammation (eg, colchicine for FMF, anakinra for periodic fever syndromes, canakinumab for CAPS, biologics for autoimmune diseases) |
| Amyloid type . | Precursor protein . | Cause . | Organ pattern . | Primary treatment* . |
|---|---|---|---|---|
| AL | Immunoglobulin light chain | Secondary to a clonal plasma cell or lymphoproliferative disorders | Heart Kidneys Liver PNS ANS Soft tissue | Chemotherapy, autologous stem cell transplantation to control underlying clonal disorder |
| ATTRwt | Wild-type transthyretin | Aging | Heart Soft tissue | Tafamidis |
| ATTRv | Mutated transthyretin | Hereditary | Heart PNS ANS GI tract | Patisiran, inotersen, vutrisiran for neuropathy Tafamidis for cardiomyopathy |
| AA | Serum amyloid A protein | Chronic inflammation | Kidneys Liver Heart ANS | Suppress inflammation (eg, colchicine for FMF, anakinra for periodic fever syndromes, canakinumab for CAPS, biologics for autoimmune diseases) |
Refers to controlling amyloidogenic precursor. Treatment of amyloidosis additionally requires strong supportive care management.
ANS, autonomic nervous system; CAPS, cryopyrin-associated periodic syndrome; FMF, familial Mediterranean fever; GI, gastrointestinal; PNS, peripheral nervous system.