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Modern Therapies in AA Amyloidosis

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Amyloid and Related Disorders

Part of the book series: Current Clinical Pathology ((CCPATH))

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Abstract

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.

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Authors and Affiliations

  1. Inflammatory Disease Section, National Human Genome Research Institute, National Institutes of Health, 10 Center Drive, 4N/208 MSC 1375, Bethesda, MD, 20892, USA

    Amanda K. Ombrello MD

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  1. Amanda K. Ombrello MD
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Correspondence to Amanda K. Ombrello MD .

Editor information

Editors and Affiliations

  1. Department of Pathology, Loyola University Medical Center, Maywood, Illinois, USA

    Maria M. Picken

  2. Department of Pathology, LSU Health Shreveport, Shreveport, Louisiana, USA

    Guillermo A. Herrera

  3. Department of Pathology and Laboratory Medicine, Department of Pathology and Laboratory Medicine, New York, New York, USA

    Ahmet Dogan

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© 2015 Springer International Publishing Switzerland

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Ombrello, A.K. (2015). Modern Therapies in AA Amyloidosis. In: Picken, M., Herrera, G., Dogan, A. (eds) Amyloid and Related Disorders. Current Clinical Pathology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-19294-9_38

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  • DOI: https://doi.org/10.1007/978-3-319-19294-9_38

  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-19293-2

  • Online ISBN: 978-3-319-19294-9

  • eBook Packages: MedicineMedicine (R0)

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