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Open Access 03-04-2024 | AA Amyloidosis | Review

AA Amyloidosis: A Contemporary View

Authors: Safak Mirioglu, Omer Uludag, Ozge Hurdogan, Gizem Kumru, Ilay Berke, Stavros A. Doumas, Eleni Frangou, Ahmet Gul

Published in: Current Rheumatology Reports

Abstract

Purpose of Review

Amyloid A (AA) amyloidosis is an organ- or life-threatening complication of chronic inflammatory disorders. Here, we review the epidemiology, causes, pathogenesis, clinical features, and diagnostic and therapeutic strategies of AA amyloidosis.

Recent Findings

The incidence of AA amyloidosis has declined due to better treatment of the underlying diseases. Histopathological examination is the gold standard of diagnosis, but magnetic resonance imaging can be used to detect cardiac involvement. There is yet no treatment option for the clearance of amyloid fibril deposits; therefore, the management strategy primarily aims to reduce serum amyloid A protein. Anti-inflammatory biologic agents have drastically expanded our therapeutic armamentarium. Kidney transplantation is preferred in patients with kidney failure, and the recurrence of amyloidosis in the allograft has become rare as transplant recipients have started to benefit from the new agents.

Summary

The management of AA amyloidosis has been considerably changed over the recent years due to the novel therapeutic options aiming to control inflammatory activity. New agents capable of clearing amyloid deposits from the tissues are still needed.

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Lachmann HJ, Goodman HJ, Gilbertson JA, Gallimore JR, Sabin CA, Gillmore JD, et al. Natural history and outcome in systemic AA amyloidosis. N Engl J Med. 2007;356(23):2361–71. https://doi.org/10.1056/NEJMoa070265.CrossRefPubMed

Papa R, Lachmann HJ. Secondary, AA, Amyloidosis. Rheum Dis Clin North Am. 2018;44(4):585–603. https://doi.org/10.1016/j.rdc.2018.06.004.CrossRefPubMed

Hemminki K, Li X, Försti A, Sundquist J, Sundquist K. Incidence and survival in non-hereditary amyloidosis in Sweden. BMC Public Health. 2012;12:974. https://doi.org/10.1186/1471-2458-12-974.CrossRefPubMedPubMedCentral

Aguirre MA, Boietti BR, Nucifora E, Sorroche PB, González Bernaldo de Quirós F, Giunta DH, et al. Incidence rate of amyloidosis in patients from a medical care program in Buenos Aires, Argentina: a prospective cohort. Amyloid. 2016;23(3):184–7. https://doi.org/10.1080/13506129.2016.1207626.

Karam S, Haidous M, Royal V, Leung N. Renal AA amyloidosis: presentation, diagnosis, and current therapeutic options: a review. Kidney Int. 2023;103(3):473–84. https://doi.org/10.1016/j.kint.2022.10.028.CrossRefPubMed

6.••

Dasari S, Theis JD, Vrana JA, Rech KL, Dao LN, Howard MT, et al. Amyloid typing by mass spectrometry in clinical practice: a comprehensive review of 16,175 samples. Mayo Clin Proc. 2020;95(9):1852–64. https://doi.org/10.1016/j.mayocp.2020.06.029. A significant study addressing the clinical use of mass spectrometry in amyloidosis.CrossRefPubMed

Real de Asúa D, Costa R, Galván JM, Filigheddu MT, Trujillo D, Cadiñanos J. Systemic AA amyloidosis: epidemiology, diagnosis, and management. Clin Epidemiol. 2014;6:369–77. https://doi.org/10.2147/clep.S39981.CrossRefPubMedPubMedCentral

Bektas M, Koca N, Oguz E, Sari S, Dagci G, Ince B, et al. Characteristics and course of patients with AA amyloidosis: single centre experience with 174 patients from Turkey. Rheumatology (Oxford). 2024;63(2):319–28. https://doi.org/10.1093/rheumatology/kead465.CrossRefPubMed

Delplanque M, Galicier L, Oziol E, Ducharme-Bénard S, Oksenhendler E, Buob D, et al. AA Amyloidosis secondary to primary immune deficiency: about 40 cases including 2 new French cases and a systematic literature review. J Allergy Clin Immunol Pract. 2021;9(2):745-52.e1. https://doi.org/10.1016/j.jaip.2020.09.023.CrossRefPubMed

10.•

Lachmann HJ. Periodic fever syndromes. Best Pract Res Clin Rheumatol. 2017;31(4):596–609. https://doi.org/10.1016/j.berh.2017.12.001. A recent review of periodic fever syndromes that are known to result in AA amyloidosis if left untreated or undertreated.CrossRefPubMed

11.

Bunker D, Gorevic P. AA amyloidosis: Mount Sinai experience, 1997–2012. Mt Sinai J Med. 2012;79(6):749–56. https://doi.org/10.1002/msj.21342.CrossRefPubMed

12.

Blank N, Hegenbart U, Dietrich S, Brune M, Beimler J, Röcken C, et al. Obesity is a significant susceptibility factor for idiopathic AA amyloidosis. Amyloid. 2018;25(1):37–45. https://doi.org/10.1080/13506129.2018.1429391.CrossRefPubMed

13.

Westermark GT, Fändrich M, Westermark P. AA amyloidosis: pathogenesis and targeted therapy. Annu Rev Pathol. 2015;10:321–44. https://doi.org/10.1146/annurev-pathol-020712-163913.CrossRefPubMed

14.

Merlini G, Bellotti V. Molecular mechanisms of amyloidosis. N Engl J Med. 2003;349(6):583–96. https://doi.org/10.1056/NEJMra023144.CrossRefPubMed

15.

Benson MD, Buxbaum JN, Eisenberg DS, Merlini G, Saraiva MJM, Sekijima Y, et al. Amyloid nomenclature 2020: update and recommendations by the International Society of Amyloidosis (ISA) nomenclature committee. Amyloid. 2020;27(4):217–22. https://doi.org/10.1080/13506129.2020.1835263.CrossRefPubMed

16.

Sack GH Jr. Serum amyloid A (SAA) proteins. Subcell Biochem. 2020;94:421–36. https://doi.org/10.1007/978-3-030-41769-7_17.CrossRefPubMed

17.

Shah C, Hari-Dass R, Raynes JG. Serum amyloid A is an innate immune opsonin for Gram-negative bacteria. Blood. 2006;108(5):1751–7. https://doi.org/10.1182/blood-2005-11-011932.CrossRefPubMed

18.

Getz GS, Krishack PA, Reardon CA. Serum amyloid A and atherosclerosis. Curr Opin Lipidol. 2016;27(5):531–5. https://doi.org/10.1097/mol.0000000000000331.CrossRefPubMed

19.

Connolly M, Mullan RH, McCormick J, Matthews C, Sullivan O, Kennedy A, et al. Acute-phase serum amyloid A regulates tumor necrosis factor α and matrix turnover and predicts disease progression in patients with inflammatory arthritis before and after biologic therapy. Arthritis Rheum. 2012;64(4):1035–45. https://doi.org/10.1002/art.33455.CrossRefPubMed

20.

Malle E, Sodin-Semrl S, Kovacevic A. Serum amyloid A: an acute-phase protein involved in tumour pathogenesis. Cell Mol Life Sci. 2009;66(1):9–26. https://doi.org/10.1007/s00018-008-8321-x.CrossRefPubMedPubMedCentral

21.

Uhlar CM, Whitehead AS. Serum amyloid A, the major vertebrate acute-phase reactant. Eur J Biochem. 1999;265(2):501–23. https://doi.org/10.1046/j.1432-1327.1999.00657.x.CrossRefPubMed

22.

Booth DR, Booth SE, Gillmore JD, Hawkins PN, Pepys MB. SAA1 alleles as risk factors in reactive systemic AA amyloidosis. Amyloid. 1998;5(4):262–5. https://doi.org/10.3109/13506129809007299.CrossRefPubMed

23.

Yilmaz E, Balci B, Kutlay S, Ozen S, Ertürk S, Oner A, et al. Analysis of the modifying effects of SAA1, SAA2 and TNF-alpha gene polymorphisms on development of amyloidosis in FMF patients. Turk J Pediatr. 2003;45(3):198–202.PubMed

24.

Sikora J, Kmochová T, Mušálková D, Pohludka M, Přikryl P, Hartmannová H, et al. A mutation in the SAA1 promoter causes hereditary amyloid A amyloidosis. Kidney Int. 2022;101(2):349–59. https://doi.org/10.1016/j.kint.2021.09.007.CrossRefPubMed

25.

Baba S, Masago SA, Takahashi T, Kasama T, Sugimura H, Tsugane S, et al. A novel allelic variant of serum amyloid A, SAA1 gamma: genomic evidence, evolution, frequency, and implication as a risk factor for reactive systemic AA-amyloidosis. Hum Mol Genet. 1995;4(6):1083–7. https://doi.org/10.1093/hmg/4.6.1083.CrossRefPubMed

26.

van der Hilst JC, Yamada T, Op den Camp HJ, van der Meer JW, Drenth JP, Simon A. Increased susceptibility of serum amyloid A 1.1 to degradation by MMP-1: potential explanation for higher risk of type AA amyloidosis. Rheumatology (Oxford). 2008;47(11):1651–4. https://doi.org/10.1093/rheumatology/ken371.CrossRefPubMed

27.

Lu J, Yu Y, Zhu I, Cheng Y, Sun PD. Structural mechanism of serum amyloid A-mediated inflammatory amyloidosis. Proc Natl Acad Sci U S A. 2014;111(14):5189–94. https://doi.org/10.1073/pnas.1322357111.CrossRefPubMedPubMedCentral

28.

Bansal A, Schmidt M, Rennegarbe M, Haupt C, Liberta F, Stecher S, et al. AA amyloid fibrils from diseased tissue are structurally different from in vitro formed SAA fibrils. Nat Commun. 2021;12(1):1013. https://doi.org/10.1038/s41467-021-21129-z.CrossRefPubMedPubMedCentral

29.

Pepys MB. Amyloidosis. Annu Rev Med. 2006;57:223–41. https://doi.org/10.1146/annurev.med.57.121304.131243.CrossRefPubMed

30.

Gharibyan AL, Zamotin V, Yanamandra K, Moskaleva OS, Margulis BA, Kostanyan IA, et al. Lysozyme amyloid oligomers and fibrils induce cellular death via different apoptotic/necrotic pathways. J Mol Biol. 2007;365(5):1337–49. https://doi.org/10.1016/j.jmb.2006.10.101.CrossRefPubMed

31.

Migita K, Agematsu K, Masumoto J, Ida H, Honda S, Jiuchi Y, et al. The contribution of SAA1 polymorphisms to Familial Mediterranean fever susceptibility in the Japanese population. PLoS ONE. 2013;8(2):e55227. https://doi.org/10.1371/journal.pone.0055227.CrossRefPubMedPubMedCentral

32.

Papa R, Doglio M, Lachmann HJ, Ozen S, Frenkel J, Simon A, et al. A web-based collection of genotype-phenotype associations in hereditary recurrent fevers from the Eurofever registry. Orphanet J Rare Dis. 2017;12(1):167. https://doi.org/10.1186/s13023-017-0720-3.CrossRefPubMedPubMedCentral

33.

Atoyan S, Hayrapetyan H, Sarkisian T, Ben-Chetrit E. MEFV and SAA1 genotype associations with clinical features of familial Mediterranean fever and amyloidosis in Armenia. Clin Exp Rheumatol. 2016;34(6 Suppl 102):72–6.PubMed

34.

Touitou I, Sarkisian T, Medlej-Hashim M, Tunca M, Livneh A, Cattan D, et al. Country as the primary risk factor for renal amyloidosis in familial Mediterranean fever. Arthritis Rheum. 2007;56(5):1706–12. https://doi.org/10.1002/art.22507.CrossRefPubMed

35.

Palladini G, Riva E, Basset M, Russo F, Milani P, Pasquinucci E, et al. Prognostication of survival and progression to dialysis in AA amyloidosis. Amyloid. 2017;24(sup1):136–7. https://doi.org/10.1080/13506129.2017.1289917.CrossRefPubMed

36.

Lane T, Pinney JH, Gilbertson JA, Hutt DF, Rowczenio DM, Mahmood S, et al. Changing epidemiology of AA amyloidosis: clinical observations over 25 years at a single national referral centre. Amyloid. 2017;24(3):162–6. https://doi.org/10.1080/13506129.2017.1342235.CrossRefPubMed

37.

Uda H, Yokota A, Kobayashi K, Miyake T, Fushimi H, Maeda A, et al. Two distinct clinical courses of renal involvement in rheumatoid patients with AA amyloidosis. J Rheumatol. 2006;33(8):1482–7.PubMed

38.

Nagata M, Shimokama T, Harada A, Koyama A, Watanabe T. Glomerular crescents in renal amyloidosis: an epiphenomenon or distinct pathology? Pathol Int. 2001;51(3):179–86. https://doi.org/10.1046/j.1440-1827.2001.01188.x.CrossRefPubMed

39.••

Kukuy OL, Beckerman P, Dinour D, Ben-Zvi I, Livneh A. Amyloid storm: acute kidney injury and massive proteinuria, rapidly progressing to end-stage kidney disease in AA amyloidosis of familial Mediterranean fever. Rheumatology (Oxford). 2021;60(7):3235–42. https://doi.org/10.1093/rheumatology/keaa772. A retrospective analysis providing great insights to the very rare “amyloid storm.”CrossRefPubMed

40.

Dahiya DS, Kichloo A, Singh J, Albosta M, Wani F. Gastrointestinal amyloidosis: a focused review. World J Gastrointest Endosc. 2021;13(1):1–12. https://doi.org/10.4253/wjge.v13.i1.1.CrossRefPubMedPubMedCentral

41.

den Braber-Ymker M, Heijker S, Lammens M, Croockewit S, Nagtegaal ID. Intestinal involvement in amyloidosis is a sequential process. Neurogastroenterol Motil. 2018;30(12):e13469. https://doi.org/10.1111/nmo.13469.CrossRef

42.

Hawkins PN. Serum amyloid P component scintigraphy for diagnosis and monitoring amyloidosis. Curr Opin Nephrol Hypertens. 2002;11(6):649–55. https://doi.org/10.1097/00041552-200211000-00013.CrossRefPubMed

43.

Ebert EC, Nagar M. Gastrointestinal manifestations of amyloidosis. Am J Gastroenterol. 2008;103(3):776–87. https://doi.org/10.1111/j.1572-0241.2007.01669.x.CrossRefPubMed

44.

Shah KB, Inoue Y, Mehra MR. Amyloidosis and the heart: a comprehensive review. Arch Intern Med. 2006;166(17):1805–13. https://doi.org/10.1001/archinte.166.17.1805.CrossRefPubMed

45.

Gertz MA, Kyle RA. Secondary systemic amyloidosis: response and survival in 64 patients. Medicine (Baltimore). 1991;70(4):246–56.CrossRefPubMed

46.

Georgin-Lavialle S, Savey L, Buob D, Bastard JP, Fellahi S, Karras A, et al. French practical guidelines for the diagnosis and management of AA amyloidosis. Rev Med Interne. 2023;44(2):62–71. https://doi.org/10.1016/j.revmed.2022.12.004.CrossRefPubMed

47.

Hazenberg BP, Bijzet J, Limburg PC, Skinner M, Hawkins PN, Butrimiene I, et al. Diagnostic performance of amyloid A protein quantification in fat tissue of patients with clinical AA amyloidosis. Amyloid. 2007;14(2):133–40. https://doi.org/10.1080/13506120701260224.CrossRefPubMed

48.

Wisniowski B, Wechalekar A. Confirming the diagnosis of amyloidosis. Acta Haematol. 2020;143(4):312–21. https://doi.org/10.1159/000508022.CrossRefPubMed

49.

van Gameren II, Hazenberg BP, Bijzet J, van Rijswijk MH. Diagnostic accuracy of subcutaneous abdominal fat tissue aspiration for detecting systemic amyloidosis and its utility in clinical practice. Arthritis Rheum. 2006;54(6):2015–21. https://doi.org/10.1002/art.21902.CrossRefPubMed

50.

Fedotov SA, Khrabrova MS, Anpilova AO, Dobronravov VA, Rubel AA. Noninvasive diagnostics of renal amyloidosis: current state and perspectives. Int J Mol Sci. 2022;23(20). https://doi.org/10.3390/ijms232012662.

51.

Said SM, Sethi S, Valeri AM, Leung N, Cornell LD, Fidler ME, et al. Renal amyloidosis: origin and clinicopathologic correlations of 474 recent cases. Clin J Am Soc Nephrol. 2013;8(9):1515–23. https://doi.org/10.2215/cjn.10491012.CrossRefPubMedPubMedCentral

52.

Sethi S, Theis JD. Pathology and diagnosis of renal non-AL amyloidosis. J Nephrol. 2018;31(3):343–50. https://doi.org/10.1007/s40620-017-0426-6.CrossRefPubMed

53.

Feitosa VA, Neves P, Jorge LB, Noronha IL, Onuchic LF. Renal amyloidosis: a new time for a complete diagnosis. Braz J Med Biol Res. 2022;55:e12284. https://doi.org/10.1590/1414-431X2022e12284.CrossRefPubMedPubMedCentral

54.

Picken MM. Modern approaches to the treatment of amyloidosis: the critical importance of early detection in surgical pathology. Adv Anat Pathol. 2013;20(6):424–39. https://doi.org/10.1097/PAP.0b013e3182a92dc3.CrossRefPubMed

55.

Dember LM. Amyloidosis-associated kidney disease. J Am Soc Nephrol. 2006;17(12):3458–71. https://doi.org/10.1681/asn.2006050460.CrossRefPubMed

56.

Taylor MS, Sidiqi H, Hare J, Kwok F, Choi B, Lee D, et al. Current approaches to the diagnosis and management of amyloidosis. Intern Med J. 2022;52(12):2046–67. https://doi.org/10.1111/imj.15974.CrossRefPubMed

57.

Gilbertson JA, Theis JD, Vrana JA, Lachmann H, Wechalekar A, Whelan C, et al. A comparison of immunohistochemistry and mass spectrometry for determining the amyloid fibril protein from formalin-fixed biopsy tissue. J Clin Pathol. 2015;68(4):314–7. https://doi.org/10.1136/jclinpath-2014-202722.CrossRefPubMed

58.

Owen-Casey MP, Sim R, Cook HT, Roufosse CA, Gillmore JD, Gilbertson JA, et al. Value of antibodies to free light chains in immunoperoxidase studies of renal biopsies. J Clin Pathol. 2014;67(8):661–6. https://doi.org/10.1136/jclinpath-2014-202231.CrossRefPubMed

59.

Fernández de Larrea C, Verga L, Morbini P, Klersy C, Lavatelli F, Foli A, et al. A practical approach to the diagnosis of systemic amyloidoses. Blood. 2015;125(14):2239–44. https://doi.org/10.1182/blood-2014-11-609883.CrossRefPubMed

60.

Sethi S, Vrana JA, Theis JD, Dogan A. Mass spectrometry based proteomics in the diagnosis of kidney disease. Curr Opin Nephrol Hypertens. 2013;22(3):273–80. https://doi.org/10.1097/MNH.0b013e32835fe37c.CrossRefPubMed

61.

Law S, Gillmore JD. When to suspect and how to approach a diagnosis of amyloidosis. Am J Med. 2022;135(Suppl 1):S2-s8. https://doi.org/10.1016/j.amjmed.2022.01.004.CrossRefPubMed

62.

Hawkins PN, Lavender JP, Pepys MB. Evaluation of systemic amyloidosis by scintigraphy with 123I-labeled serum amyloid P component. N Engl J Med. 1990;323(8):508–13. https://doi.org/10.1056/nejm199008233230803.CrossRefPubMed

63.

Gertz MA, Comenzo R, Falk RH, Fermand JP, Hazenberg BP, Hawkins PN, et al. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th International Symposium on Amyloid and Amyloidosis, Tours, France, 18–22 April 2004. Am J Hematol. 2005;79(4):319–28. https://doi.org/10.1002/ajh.20381.CrossRefPubMed

64.

Perugini E, Rapezzi C, Piva T, Leone O, Bacchi-Reggiani L, Riva L, et al. Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance. Heart. 2006;92(3):343–9. https://doi.org/10.1136/hrt.2005.061911.CrossRefPubMed

65.

Kidney Disease: Improving Global Outcomes (KDIGO) CKD Work Group. KDIGO 2024 clinical practice guideline for the evaluation and management of chronic kidney disease. Kidney Int. 2024;105(4S):S117-S314. https://doi.org/10.1016/j.kint.2023.10.018.

66.

Weinreb JC, Rodby RA, Yee J, Wang CL, Fine D, McDonald RJ, et al. Use of intravenous gadolinium-based contrast media in patients with kidney disease: consensus statements from the American College of Radiology and the National Kidney Foundation. Radiology. 2021;298(1):28–35. https://doi.org/10.1148/radiol.2020202903.CrossRefPubMed

67.

Flett AS, Hayward MP, Ashworth MT, Hansen MS, Taylor AM, Elliott PM, et al. Equilibrium contrast cardiovascular magnetic resonance for the measurement of diffuse myocardial fibrosis: preliminary validation in humans. Circulation. 2010;122(2):138–44. https://doi.org/10.1161/circulationaha.109.930636.CrossRefPubMed

68.

Livneh A, Zemer D, Langevitz P, Shemer J, Sohar E, Pras M. Colchicine in the treatment of AA and AL amyloidosis. Semin Arthritis Rheum. 1993;23(3):206–14. https://doi.org/10.1016/s0049-0172(05)80042-3.CrossRefPubMed

69.

Berglund K, Thysell H, Keller C. Results, principles and pitfalls in the management of renal AA-amyloidosis; a 10–21 year followup of 16 patients with rheumatic disease treated with alkylating cytostatics. J Rheumatol. 1993;20(12):2051–7.PubMed

70.

Nakamura T, Yamamura Y, Tomoda K, Tsukano M, Shono M, Baba S. Efficacy of cyclophosphamide combined with prednisolone in patients with AA amyloidosis secondary to rheumatoid arthritis. Clin Rheumatol. 2003;22(6):371–5. https://doi.org/10.1007/s10067-003-0763-9.CrossRefPubMed

71.

Gillmore JD, Lovat LB, Persey MR, Pepys MB, Hawkins PN. Amyloid load and clinical outcome in AA amyloidosis in relation to circulating concentration of serum amyloid A protein. Lancet. 2001;358(9275):24–9. https://doi.org/10.1016/s0140-6736(00)05252-1.CrossRefPubMed

72.

Kidney Disease: Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney Int. 2021;100(4S):S1-S276. https://doi.org/10.1016/j.kint.2021.05.021.

73.

Zemer D, Revach M, Pras M, Modan B, Schor S, Sohar E, et al. A controlled trial of colchicine in preventing attacks of familial mediterranean fever. N Engl J Med. 1974;291(18):932–4. https://doi.org/10.1056/nejm197410312911803.CrossRefPubMed

74.

Leung YY, Yao Hui LL, Kraus VB. Colchicine–update on mechanisms of action and therapeutic uses. Semin Arthritis Rheum. 2015;45(3):341–50. https://doi.org/10.1016/j.semarthrit.2015.06.013.CrossRefPubMedPubMedCentral

75.

Zemer D, Pras M, Sohar E, Modan M, Cabili S, Gafni J. Colchicine in the prevention and treatment of the amyloidosis of familial Mediterranean fever. N Engl J Med. 1986;314(16):1001–5. https://doi.org/10.1056/nejm198604173141601.CrossRefPubMed

76.

Livneh A, Zemer D, Langevitz P, Laor A, Sohar E, Pras M. Colchicine treatment of AA amyloidosis of familial Mediterranean fever. An analysis of factors affecting outcome. Arthritis Rheum. 1994;37(12):1804–11. https://doi.org/10.1002/art.1780371215.CrossRefPubMed

77.

Unverdi S, Inal S, Ceri M, Unverdi H, Batgi H, Tuna R, et al. Is colchicine therapy effective in all patients with secondary amyloidosis? Ren Fail. 2013;35(8):1071–4. https://doi.org/10.3109/0886022x.2013.811345.CrossRefPubMed

78.

Gottenberg JE, Merle-Vincent F, Bentaberry F, Allanore Y, Berenbaum F, Fautrel B, et al. Anti-tumor necrosis factor alpha therapy in fifteen patients with AA amyloidosis secondary to inflammatory arthritides: a followup report of tolerability and efficacy. Arthritis Rheum. 2003;48(7):2019–24. https://doi.org/10.1002/art.11163.CrossRefPubMed

79.

Fernández-Nebro A, Olivé A, Castro MC, Varela AH, Riera E, Irigoyen MV, et al. Long-term TNF-alpha blockade in patients with amyloid A amyloidosis complicating rheumatic diseases. Am J Med. 2010;123(5):454–61. https://doi.org/10.1016/j.amjmed.2009.11.010.CrossRefPubMed

80.

Esatoglu SN, Hatemi G, Ugurlu S, Gokturk A, Tascilar K, Ozdogan H. Long-term follow-up of secondary amyloidosis patients treated with tumor necrosis factor inhibitor therapy: a STROBE-compliant observational study. Medicine (Baltimore). 2017;96(34):e7859. https://doi.org/10.1097/md.0000000000007859.CrossRefPubMed

81.

Courties A, Grateau G, Philippe P, Flipo RM, Astudillo L, Aubry-Rozier B, et al. AA amyloidosis treated with tocilizumab: case series and updated literature review. Amyloid. 2015;22(2):84–92. https://doi.org/10.3109/13506129.2014.1002031.CrossRefPubMed

82.

Okuda Y, Ohnishi M, Matoba K, Jouyama K, Yamada A, Sawada N, et al. Comparison of the clinical utility of tocilizumab and anti-TNF therapy in AA amyloidosis complicating rheumatic diseases. Mod Rheumatol. 2014;24(1):137–43. https://doi.org/10.3109/14397595.2013.854048.CrossRefPubMed

83.

Serra I, Oller B, Mañosa M, Naves JE, Zabana Y, Cabré E, et al. Systemic amyloidosis in inflammatory bowel disease: retrospective study on its prevalence, clinical presentation, and outcome. J Crohns Colitis. 2010;4(3):269–74. https://doi.org/10.1016/j.crohns.2009.11.009.CrossRefPubMed

84.••

Ben-Zvi I, Kukuy O, Giat E, Pras E, Feld O, Kivity S, et al. Anakinra for colchicine-resistant familial Mediterranean fever: a randomized, double-blind, placebo-controlled trial. Arthritis Rheumatol. 2017;69(4):854–62. https://doi.org/10.1002/art.39995. Randomized controlled trial of anakinra in patients with colchicine-resistant FMF which is a known cause of AA amyloidosis, especially in the Mediterranean basin.CrossRefPubMed

85.

Bodar EJ, Kuijk LM, Drenth JP, van der Meer JW, Simon A, Frenkel J. On-demand anakinra treatment is effective in mevalonate kinase deficiency. Ann Rheum Dis. 2011;70(12):2155–8. https://doi.org/10.1136/ard.2011.149922.CrossRefPubMed

86.

Neven B, Marvillet I, Terrada C, Ferster A, Boddaert N, Couloignier V, et al. Long-term efficacy of the interleukin-1 receptor antagonist anakinra in ten patients with neonatal-onset multisystem inflammatory disease/chronic infantile neurologic, cutaneous, articular syndrome. Arthritis Rheum. 2010;62(1):258–67. https://doi.org/10.1002/art.25057.CrossRefPubMed

87.

Gattorno M, Pelagatti MA, Meini A, Obici L, Barcellona R, Federici S, et al. Persistent efficacy of anakinra in patients with tumor necrosis factor receptor-associated periodic syndrome. Arthritis Rheum. 2008;58(5):1516–20. https://doi.org/10.1002/art.23475.CrossRefPubMed

88.

Lovell DJ, Giannini EH, Reiff AO, Kimura Y, Li S, Hashkes PJ, et al. Long-term safety and efficacy of rilonacept in patients with systemic juvenile idiopathic arthritis. Arthritis Rheum. 2013;65(9):2486–96. https://doi.org/10.1002/art.38042.CrossRefPubMed

89.

Hashkes PJ, Spalding SJ, Giannini EH, Huang B, Johnson A, Park G, et al. Rilonacept for colchicine-resistant or -intolerant familial Mediterranean fever: a randomized trial. Ann Intern Med. 2012;157(8):533–41. https://doi.org/10.7326/0003-4819-157-8-201210160-00003.CrossRefPubMed

90.••

De Benedetti F, Gattorno M, Anton J, Ben-Chetrit E, Frenkel J, Hoffman HM, et al. Canakinumab for the treatment of autoinflammatory recurrent fever syndromes. N Engl J Med. 2018;378(20):1908–19. https://doi.org/10.1056/NEJMoa1706314. Landmark study of canakinumab for the treatment of periodic fever syndromes.CrossRefPubMed

91.

Ruperto N, Brunner HI, Quartier P, Constantin T, Wulffraat N, Horneff G, et al. Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012;367(25):2396–406. https://doi.org/10.1056/NEJMoa1205099.CrossRefPubMed

92.

Hoffman HM, Throne ML, Amar NJ, Sebai M, Kivitz AJ, Kavanaugh A, et al. Efficacy and safety of rilonacept (interleukin-1 Trap) in patients with cryopyrin-associated periodic syndromes: results from two sequential placebo-controlled studies. Arthritis Rheum. 2008;58(8):2443–52. https://doi.org/10.1002/art.23687.CrossRefPubMed

93.

Goldbach-Mansky R, Shroff SD, Wilson M, Snyder C, Plehn S, Barham B, et al. A pilot study to evaluate the safety and efficacy of the long-acting interleukin-1 inhibitor rilonacept (interleukin-1 Trap) in patients with familial cold autoinflammatory syndrome. Arthritis Rheum. 2008;58(8):2432–42. https://doi.org/10.1002/art.23620.CrossRefPubMedPubMedCentral

94.

Sack GH Jr. Serum amyloid A - a review. Mol Med. 2018;24(1):46. https://doi.org/10.1186/s10020-018-0047-0.CrossRefPubMedPubMedCentral

95.

Dember LM, Hawkins PN, Hazenberg BP, Gorevic PD, Merlini G, Butrimiene I, et al. Eprodisate for the treatment of renal disease in AA amyloidosis. N Engl J Med. 2007;356(23):2349–60. https://doi.org/10.1056/NEJMoa065644.CrossRefPubMed

96.

(2016) Bellus Health: Phase 3 Results of Kiacta for AA Amyloidosis. https://www.drugdiscoverytrends.com/bellus-health-phase-3-results-of-kiacta-for-aa-amyloidosis/. Accessed February 9, 2024.

97.

Tennent GA, Lovat LB, Pepys MB. Serum amyloid P component prevents proteolysis of the amyloid fibrils of Alzheimer disease and systemic amyloidosis. Proc Natl Acad Sci U S A. 1995;92(10):4299–303. https://doi.org/10.1073/pnas.92.10.4299.CrossRefPubMedPubMedCentral

98.

Gillmore JD, Tennent GA, Hutchinson WL, Gallimore JR, Lachmann HJ, Goodman HJ, et al. Sustained pharmacological depletion of serum amyloid P component in patients with systemic amyloidosis. Br J Haematol. 2010;148(5):760–7. https://doi.org/10.1111/j.1365-2141.2009.08036.x.CrossRefPubMed

99.•

Richards DB, Cookson LM, Berges AC, Barton SV, Lane T, Ritter JM, et al. Therapeutic clearance of amyloid by antibodies to serum amyloid p component. N Engl J Med. 2015;373(12):1106–14. https://doi.org/10.1056/NEJMoa1504942. Phase 1 trial of anti-SAP antibodies that are aimed to clear amyloid deposits from the affected tissues.CrossRefPubMed

100.

Richards DB, Cookson LM, Barton SV, Liefaard L, Lane T, Hutt DF, et al. Repeat doses of antibody to serum amyloid P component clear amyloid deposits in patients with systemic amyloidosis. Sci Transl Med. 2018;10(422). https://doi.org/10.1126/scitranslmed.aan3128.

101.

Richards D, Bamford M, Liefaard L, Haque N, Lewis G, Storey J, et al. Identification, preclinical profile, and clinical proof of concept of an orally bioavailable pro-drug of miridesap. Br J Pharmacol. 2020;177(8):1853–64. https://doi.org/10.1111/bph.14956.CrossRefPubMedPubMedCentral

102.

Kluve-Beckerman B, Du Hardwick, L, Benson MD. AA amyloidosis: potential therapy with antisense oligonucleotides. Amyloid. 2011;18(Suppl 1):200–2. https://doi.org/10.3109/13506129.2011.574354075.CrossRefPubMed

103.

Bollée G, Guery B, Joly D, Snanoudj R, Terrier B, Allouache M, et al. Presentation and outcome of patients with systemic amyloidosis undergoing dialysis. Clin J Am Soc Nephrol. 2008;3(2):375–81. https://doi.org/10.2215/cjn.02470607.CrossRefPubMedPubMedCentral

104.

Gursu M, Yelken B, Caliskan Y, Kazancioglu R, Yazici H, Kilicaslan I, et al. Outcome of patients with amyloidosis after renal transplantation: a single-center experience. Int J Artif Organs. 2012;35(6):444–9. https://doi.org/10.5301/ijao.5000091.CrossRefPubMed

105.

Sarihan I, Caliskan Y, Mirioglu S, Ozluk Y, Senates B, Seyahi N, et al. Amyloid A amyloidosis after renal transplantation: an important cause of mortality. Transplantation. 2020;104(8):1703–11. https://doi.org/10.1097/tp.0000000000003043.CrossRefPubMed

106.••

Schwarz C, Georgin-Lavialle S, Lombardi Y, Marion O, Jambon F, Legendre C, et al. Kidney transplantation in patients with AA amyloidosis: outcomes in a French multicenter cohort. Am J Kidney Dis. 2023. https://doi.org/10.1053/j.ajkd.2023.07.020. A very recent report showing the decline of recurrent AA amyloidosis in allografts due to better diagnosis and treatment of the underlying disorders.

107.

Haq A, Hussain S, Meskat B, Mohan P, Conlon P, Hickey DP. Complications of renal transplantation in patients with amyloidosis. Transplant Proc. 2007;39(1):120–4. https://doi.org/10.1016/j.transproceed.2006.10.021.CrossRefPubMed

108.

Garrouste C, Anglicheau D, Kamar N, Bachelier C, Rivalan J, Pereira B, et al. Anti-TNFα therapy for chronic inflammatory disease in kidney transplant recipients: clinical outcomes. Medicine (Baltimore). 2016;95(41): e5108. https://doi.org/10.1097/md.0000000000005108.CrossRefPubMed

109.••

Mirioglu S, Dirim AB, Bektas M, Demir E, Tor YB, Ozluk Y, et al. Efficacy and safety of interleukin-1 blockers in kidney transplant recipients with familial Mediterranean fever: a propensity score-matched cohort study. Nephrol Dial Transplant. 2023;38(5):1327–36. https://doi.org/10.1093/ndt/gfac335. The largest study on the efficacy and safety of IL-1 blockers in kidney transplant recipients suffering from FMF and AA amyloidosis.CrossRefPubMed

110.

Wason S, Digiacinto JL, Davis MW. Effect of cyclosporine on the pharmacokinetics of colchicine in healthy subjects. Postgrad Med. 2012;124(4):189–96. https://doi.org/10.3810/pgm.2012.07.2579.CrossRefPubMed

Title: AA Amyloidosis: A Contemporary View
Authors: Safak Mirioglu
Omer Uludag
Ozge Hurdogan
Gizem Kumru
Ilay Berke
Stavros A. Doumas
Eleni Frangou
Ahmet Gul
Publication date: 03-04-2024
Publisher: Springer US
Keywords: AA Amyloidosis
Amyloidosis
Familial Mediterranean Fever
Autoinflammatory Diseases
Arthritis
Published in: Current Rheumatology Reports
Print ISSN: 1523-3774
Electronic ISSN: 1534-6307
DOI: https://doi.org/10.1007/s11926-024-01147-8

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