AA amyloidosis

AA amyloidosis
SpecialtyRheumatology

AA amyloidosis is a form of amyloidosis, a disease characterized by the abnormal deposition of fibers of insoluble protein in the extracellular space of various tissues and organs. In AA amyloidosis, the deposited protein is serum amyloid A protein (SAA), an acute-phase protein which is normally soluble and whose plasma concentration is highest during inflammation.[1]

Causes

AA amyloidosis is a complication of a number of inflammatory diseases and infections,[2] although only a small portion of patients with these conditions will go on to develop AA amyloidosis. The most common presentation of AA amyloidosis is renal in nature, including proteinuria, nephrotic syndrome and progressive development of chronic kidney disease leading to end stage kidney disease (ESKD) and need for renal replacement therapy (e.g. dialysis or kidney transplantation).[3] A natural history study of AA amyloidosis patients reported a number of conditions associated with AA amyloidosis:[1]

Symptoms

Signs and symptoms of Amyloidosis can vary depending on the affected organ. AA amyloidosis is commonly found in organs like the kidneys, liver, and stomach. If the kidneys are affected, an individual may see swelling in the legs, reduced urination, and at times excessive bubbles in the urine. Amyloid proteins in the kidneys can also lead to high cholesterol. [8] Other organs like the stomach may result in bleeding, constipation, etc. [9]

Pathology

In a healthy individual, the median plasma concentration of SAA is 3 mg per liter.[10] This can increase to over 2000 mg per liter during an acute phase response and a sustained overproduction of SAA is required for the creation of the AA deposits that define AA amyloidosis.[11] High levels of SAA, however, is not a sufficient condition for the development of systemic AA amyloidosis and it remains unclear what triggers the accumulation of AA.[12]

The AA protein is mainly deposited in the liver, spleen and kidney, and AA amyloidosis can lead to nephrotic syndrome and ESRD.[13][14] Natural history studies show, however, that it is the kidney involvement that drives the progression of the disease. In general, old age, reduced serum albumin concentration, end stage kidney failure, and sustained elevated SAA concentration are all associated with poor prognosis.[15]

There are currently no approved treatments for systemic AA amyloidosis.[13] The current standard of care includes treatments for the underlying inflammatory disease with anti-inflammatory drugs, immunosuppressive agents or biologics. AA amyloidosis patients are also receiving treatments to slow down the decline of their renal function, such as angiotensin II receptor blockers or angiotensin converting enzyme inhibitors.[16]

Transmission of amyloidosis

There is evidence that eating amyloid fibers may lead to amyloidosis. This evidence is based on studies in cattle, chickens, mice, and cheetahs.[17] Thus, in a sense, SAA amyloidosis may be considered a contagious disease, although whether this occurs or is important in the development of naturally occurring amyloidosis remains unknown. Nevertheless, because amyloid fibers can be detected in muscle in low amounts, it raises some concern about whether people could develop amyloidosis as a result of ingesting meat from an animal with the disease.[17]

References

  1. 1 2 Lachmann HJ, Goodman HJ, Gilbertson JA, et al. (June 2007). "Natural history and outcome in systemic AA amyloidosis" (PDF). New England Journal of Medicine. 356 (23): 2361–71. doi:10.1056/NEJMoa070265. PMID 17554117. Archived from the original (PDF) on 2014-01-09.
  2. ↑ Chapter 5 in: Mitchell, Richard Sheppard; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson (2007). Robbins Basic Pathology. Philadelphia: Saunders. ISBN 978-1-4160-2973-1. 8th edition.
  3. ↑ Richa Dhawan; Mohammed Mubashir Ahmed; Eisha Mubashir; Joel N Buxbaum; Ratinder J Kaur. "AA (Inflammatory) Amyloidosis Clinical Presentation". {{cite journal}}: Cite journal requires |journal= (help)
  4. ↑ Jung, Oliver; Haack, Hans Stefan; Buettner, Maike; Betz, Christoph; Stephan, Christoph; Gruetzmacher, Peter; Amann, Kerstin; Bickel, Markus (2012). "Renal AA-amyloidosis in intravenous drug users – a role for HIV-infection?". BMC Nephrology. 13: 151. doi:10.1186/1471-2369-13-151. PMC 3519698. PMID 23171281.
  5. ↑ d'Ythurbide, G; Kerrou, K; Brocheriou, I; Hertig, A (2012). "Reactive amyloidosis complicated by end-stage renal disease 28 years after liquid silicone injection in the buttocks". BMJ Case Reports. 2012: bcr2012006803. doi:10.1136/bcr-2012-006803. PMC 4543521. PMID 23035166.
  6. ↑ Emekli, U; Tümerdem, B; Demiryont, M (2002). "Rupture of a silicone gel mammary prosthesis and amyloidosis: A case report". Aesthetic Plastic Surgery. 26 (5): 383–7. doi:10.1007/s00266-002-2022-x. PMID 12432480. S2CID 6865930.
  7. ↑ Goldman, A. B.; Bansal, M (1996). "Amyloidosis and silicone synovitis: Updated classification, updated pathophysiology, and synovial articular abnormalities". Radiologic Clinics of North America. 34 (2): 375–94, xi. PMID 8633122.
  8. ↑ "AA Amyloidosis". Amyloidosis Foundation. Retrieved 9 January 2022.
  9. ↑ "Amyloidosis: AA". Cleveland Clinic. Retrieved 9 January 2022.
  10. ↑ Biasucci LM, Liuzzo G, Grillo RL, Caligiuri G, Rebuzzi AG, Buffon A, Summaria F, Ginnetti F, Fadda G, Maseri A (February 1999). "Elevated levels of C-reactive protein at discharge in patients with unstable angina predict recurrent instability". Institute of Cardiology, Catholic University of the Sacred Heart. 99 (7): 855–60. doi:10.1161/01.cir.99.7.855. PMID 10027805.
  11. ↑ Familial Mediterranean Fever. Springer. 2015-03-19. ISBN 978-3319146157.
  12. ↑ Diego Real de Asúa; Ramón Costa; Jose María Galván; María Teresa Filigheddu; Davinia Trujillo; Julen Cadiñanos (2014). "Systemic AA amyloidosis: epidemiology, diagnosis, and management". Clinical Epidemiology. 6: 369–77. doi:10.2147/CLEP.S39981. PMC 4218891. PMID 25378951.
  13. 1 2 "AA (Inflammatory) Amyloidosis". Medscape Reference. 2019-02-02.
  14. ↑ "AA Amyloidosis". BU Amyloid Treatment & Research Program.
  15. ↑ Daisuke Katagiri; Eisei Noiri; Fumihiko Hinoshita (2013). "Multiple Myeloma and Kidney Disease". The Scientific World Journal. 2013: 1–9. doi:10.1155/2013/487285. PMC 3826468. PMID 24288486.
  16. ↑ Fernández-Nebro A (2005). "Treatment of rheumatic inflammatory disease in 25 patients with secondary amyloidosis using tumor necrosis factor alpha antagonists". Am J Med. 118 (5): 552–6. doi:10.1016/j.amjmed.2005.01.028. PMID 15866260. Retrieved 12 June 2015.
  17. 1 2 Murakami, T; Ishiguro N; Higuchi K (March 2014). "Transmission of Systemic AA Amyloidosis in Animals". Veterinary Pathology. 51 (2): 363–371. doi:10.1177/0300985813511128. PMID 24280941.

(www.AmyloidAware.com ) Booklet and explanatory video explaining the difference between the types of amyloidosis. Written by doctors at Mayo Clinic, Boston University, Indiana University and others

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