Modern Therapies in AA Amyloidosis

  • Amanda K. OmbrelloEmail author
Part of the Current Clinical Pathology book series (CCPATH)


AA amyloidosis is a condition that develops in patients who have long-standing inflammatory conditions. The chronic inflammatory state leads to misfolding of the AA amyloid protein and resultant deposition in tissues. In the twenty-first century, rheumatic diseases such as rheumatoid arthritis, ankylosing spondylitis, and systemic juvenile arthritis as well as autoinflammatory diseases such as familial Mediterranean fever (FMF) and tumor necrosis factor receptor associated periodic syndrome (TRAPS) and the group of diseases known as the cryopyrinopathies comprise a major portion of AA amyloidosis cases both in the United States and in Europe. Currently, there is not any established therapy for AA amyloidosis and treatment is targeted at reducing the underlying inflammatory condition. The development of the tumor necrosis factor inhibitors as well as the IL-1 and IL-6 antagonists has been paramount in the treatment of autoimmune and autoinflammatory diseases and there are case reports of their efficacy in treating patients with AA amyloidosis. A clinical trial is currently under way using a medication that attempts to prevent AA amyloid deposition.


AA amyloidosis Rheumatic disease Autoinflammatory disease IL-1-mediated disease Inflammatory bowel disease Biologic medications Anti-tumor necrosis factor medications IL-1 antagonists Etanercept Adalimumab Infliximab Anakinra Rilonacept Canakinumab Tocilizumab Eprodisate disodium 


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Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  1. 1.Inflammatory Disease SectionNational Human Genome Research Institute, National Institutes of HealthBethesdaUSA

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