Atrial infiltration is present and frequently causes contractile dysfunction. [ahajournals.org]

• Inflammation

  They found that the mutation reduces activity of the PSMB8 enzyme within the immune cells and affects normal processing of antigens, resulting in inflammation. [hcplive.com]

  […] multifactorial, resulting from a combination of factors including extracellular deposition of amyloid in the parenchymal tissue leading to mechanical disruption of tissue structure, as well as proteotoxicity of the fibrils or prefibrillar proteins leading to inflammation [ahajournals.org]

• Amyloidosis

  […] of amyloidogenic light chain fragments prone to improper aggregation.19 Other less common forms of CA encountered clinically include AA amyloidosis, dialyses associated amyloidosis, and isolated atrial amyloidosis. [ahajournals.org]

• Heart Failure

  Clinical Manifestations and Diagnostic Evaluation of Cardiac Amyloidosis AL (light chain amyloidosis) TTR (transthyretin amyloidosis) Clinical features Heart failure with a preserved ejection fraction Right heart failure (JVP, hepatomegaly, and edema) [ahajournals.org]

• Joint Stiffness

  Researchers have identified a gene responsible for a rare disease leads to progressive loss of fat and muscle, and joint stiffness. and a gene responsible for a rare disease that results in severe joint stiffness, muscle loss, anemia and panniculitis-induced [hcplive.com]

• Peripheral Neuropathy

  These patients also often have marked cardiac involvement at presentation with concomitant peripheral neuropathy. [ahajournals.org]

[…] initiation, outcomes of these treatment combinations remain poor. [ahajournals.org]

Immunoglobulin light chain amyloidosis: 2018 Update on diagnosis, prognosis, and treatment.Am J Hematol. 2018; 93:1169–1180. doi: 10.1002/ajh.25149CrossrefMedlineGoogle Scholar 70. [ahajournals.org]

The pathology and changing epidemiology of dialysis-related cardiac beta-2 microglobulin amyloidosis.Cardiovasc Pathol. 2019; 42:30–35. doi: 10.1016/j.carpath.2019.05.002CrossrefMedlineGoogle Scholar 22. [ahajournals.org]

Median survival 3–12 y Pathophysiology Despite originating from different precursor proteins, the basic mechanisms underlying amyloid pathogenesis is similar in that the capability of a protein to become amyloidogenic lies in its ability to acquire >1 [ahajournals.org]

The next step, Garg said, is to determine the best type of therapy and whether it is possible to prevent the disease or lessen the severity of some of its symptoms. "This is a good start," Garg said. [hcplive.com]

Additionally, it prevents proteins from misfolding and aggregating and removes proteins that are misassembled. [ahajournals.org]