Table 1 Most common types of amyloidosis, clinical findings, and treatment
From: Uncovering the knowledge about systemic amyloidosis relevant to the rheumatologists
AA | AL | ATTRv | ATTRwt | AB2M | |
|---|---|---|---|---|---|
Protein that causes amyloid deposits | serum amyloid A (SAA) protein | kappa or lambda light chains | mutated transthyretin misfolding | age-related transthyretin misfolding | beta2-microglobulin |
Clinical features | renal involvement with edema, isolated proteinuria, and/or renal failure | heart failure, spontaneous ecchymosis and periorbital purpura, macroglossia, soft-tissue infiltration in the shoulders, bilateral carpal tunnel syndrome and GI involvement | polyneuropathy, autonomic dysfunction, heart failure, cardiac arrhythmias, bilateral carpal tunnel syndrome | heart failure, autonomic dysfunction, bilateral carpal tunnel syndrome, acquired lumbar spinal stenosis, and rupture of the long biceps tendon | cystic bone lesions, involvement of the cervical spine, wrists, and shoulders, and infiltration of musculoskeletal soft tissues with bilateral carpal tunnel syndrome |
Treatment | biological agents- anti-TNF, anti-IL6 and anti-IL1 | Hematopoietic transplantation, combination of chemotherapy, steroids, bortezomib, daratumumab | transthyretin stabilizers, oligonucleotide therapy, liver transplantation | transthyretin stabilizers | hemodialysis with high-flux biocompatible membranes, which remove beta2-microglobulins effectively, and dialysates that minimize inflammation and production of beta2-microglobulin |