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Secondary amyloidosis medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Roukoz A. Karam, M.D.[2]

Overview

Overview

Medical or surgical treatment of the underlying chronic infection or inflammatory disease is recommended among all patients who develop AA amyloidosis.

Medical Therapy

Medical Therapy

  • Medical or surgical treatment of the underlying chronic infection or inflammatory disease is recommended among all patients who develop AA amyloidosis.[1]
  • Aggressively treating the disease that is causing the excess amyloid protein can improve symptoms and slow down or halt the progression of the disease.
  • Complications such as heart failure, renal failure, and other problems can sometimes be treated, when needed.
  • Although the choice of therapy depends on the underlying cause of chronic inflammation, the aim is always to suppress production of SAA to within the normal range.
  • Examples of treatments for the commonest disorders underlying AA amyloidosis, adapted from Rheumatic Diseases Clinics of North America:[2]
Underlying Condition Treatment Options Examples
Inflammatory arthritis Conventional disease-modifying agents

Other immunosuppressant agents

Biologic agents

Periodic fevers On-demand agents

Colchicine (for familial mediterranean fever)

Biologic agents

Inflammatory bowel disease Conventional disease-modifying agents

Biologic agents

Antibiotics

Biologic agents

Surgery

  • ileo-cecal resection and primary reconstruction
Immunodeficiency Immunoglobulins

    Antibiotics

    Chronic infections Antibiotics and surgery

      Physiotherapy (in case of bronchiectasis)

        Immunodeficiency Immunoglobulins

          Antibiotics

          Neoplasia Chemotherapy and surgery

          Varies according to type of cancer

          Biologic agents (in Castleman disease)

          • Long-term inflammatory control can be accompanied by gradual regression of amyloid deposits and improvement in renal function.
          • Currently a second clinical trial is in progress in order to evaluate a targeted inhibitor molecule, Kiacta, in the management of secondary amyloidosis.[3]
          • Novel therapies aimed at promoting clearance of existing amyloid deposits soon may be an effective treatment approach.


          References

          References

          1. Desport E, Bridoux F, Sirac C, Delbes S, Bender S, Fernandez B; et al. (2012). “Al amyloidosis”. Orphanet J Rare Dis. 7: 54. doi:10.1186/1750-1172-7-54. PMC 3495844. PMID 22909024.
          2. Papa R, Lachmann HJ (2018). “Secondary, AA, Amyloidosis”. Rheum Dis Clin North Am. 44 (4): 585–603. doi:10.1016/j.rdc.2018.06.004. PMID 30274625.
          3. Hazenberg BP, Bijzet J, Limburg PC, Skinner M, Hawkins PN, Butrimiene I; et al. (2007). “Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis”. Amyloid. 14 (2): 133–40. doi:10.1080/13506120701260224. PMID 17577686.

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