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AA amyloidosis

MedGen UID:
782429
Concept ID:
C3536715
Disease or Syndrome
Synonym: Secondary amyloidosis
SNOMED CT: Amyloid A amyloidosis (274945004); AA amyloidosis (274945004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:4000041
Monarch Initiative: MONDO:0019439
Orphanet: ORPHA85445

Definition

Extracellular tissue deposition of fibrils that are composed of fragments of and/or intact serum amyloid A protein, a hepatic acute phase reactant. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAA amyloidosis

Conditions with this feature

TNF receptor-associated periodic fever syndrome (TRAPS)
MedGen UID:
226899
Concept ID:
C1275126
Disease or Syndrome
TNF receptor-associated periodic fever syndrome (TRAPS) is characterized by episodes of inflammation typically occurring every four to six weeks and lasting between five and 25 days. Flares may be prompted by stress, infection, trauma, hormonal changes, and vaccination. Symptoms may include fever, abdominal pain, arthralgia, myalgia, migratory rash, and eye inflammation, with variable severity. Symptoms often begin in early childhood (median age 4.3 years), though symptom onset can occur later in life. During a flare, acute-phase reactants such as C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and serum amyloid A are typically elevated. Generally, acute-phase reactants stabilize between flares but may remain somewhat elevated even in the absence of clinical symptoms. AA amyloidosis, the most severe sequela of TRAPS, can largely be avoided with adequate treatment. Proteinuria and kidney failure occur in 80%-90% of affected individuals with amyloidosis, while intestinal, thyroid, myocardium, liver, and spleen deposits are less common.

Professional guidelines

PubMed

Allinovi M, Trivioli G, Gaudio C, L'Imperio V, Rauf MU, Gillmore JD
Nephrol Dial Transplant 2025 Sep 29;40(10):1826-1837. doi: 10.1093/ndt/gfaf042. PMID: 40036358
De Matteis A, Bindoli S, De Benedetti F, Carmona L, Fautrel B, Mitrovic S
Ann Rheum Dis 2024 Nov 14;83(12):1748-1761. doi: 10.1136/ard-2024-225853. PMID: 39317414Free PMC Article
Tan SY, Pepys MB, Hawkins PN
Am J Kidney Dis 1995 Aug;26(2):267-85. doi: 10.1016/0272-6386(95)90647-9. PMID: 7645531

Recent clinical studies

Etiology

De Matteis A, Bindoli S, De Benedetti F, Carmona L, Fautrel B, Mitrovic S
Ann Rheum Dis 2024 Nov 14;83(12):1748-1761. doi: 10.1136/ard-2024-225853. PMID: 39317414Free PMC Article
Kozycki CT, Kodati S, Huryn L, Wang H, Warner BM, Jani P, Hammoud D, Abu-Asab MS, Jittayasothorn Y, Mattapallil MJ, Tsai WL, Ullah E, Zhou P, Tian X, Soldatos A, Moutsopoulos N, Kao-Hsieh M, Heller T, Cowen EW, Lee CR, Toro C, Kalsi S, Khavandgar Z, Baer A, Beach M, Long Priel D, Nehrebecky M, Rosenzweig S, Romeo T, Deuitch N, Brenchley L, Pelayo E, Zein W, Sen N, Yang AH, Farley G, Sweetser DA, Briere L, Yang J, de Oliveira Poswar F, Schwartz IVD, Silva Alves T, Dusser P, Koné-Paut I, Touitou I, Titah SM, van Hagen PM, van Wijck RTA, van der Spek PJ, Yano H, Benneche A, Apalset EM, Jansson RW, Caspi RR, Kuhns DB, Gadina M, Takada H, Ida H, Nishikomori R, Verrecchia E, Sangiorgi E, Manna R, Brooks BP, Sobrin L, Hufnagel RB, Beck D, Shao F, Ombrello AK, Aksentijevich I, Kastner DL; Undiagnosed Diseases Network
Ann Rheum Dis 2022 Oct;81(10):1453-1464. Epub 2022 Jul 22 doi: 10.1136/annrheumdis-2022-222629. PMID: 35868845Free PMC Article
Deshayes S, Aouba A, Grateau G, Georgin-Lavialle S
Int J Clin Pract 2021 Jun;75(6):e13966. Epub 2021 Jan 20 doi: 10.1111/ijcp.13966. PMID: 33368925
Ozdogan H, Ugurlu S
Presse Med 2019 Feb;48(1 Pt 2):e61-e76. Epub 2019 Jan 25 doi: 10.1016/j.lpm.2018.08.014. PMID: 30686512
Bansal R, Syed U, Walfish J, Aron J, Walfish A
Curr Gastroenterol Rep 2018 Mar 26;20(3):11. doi: 10.1007/s11894-018-0616-y. PMID: 29582184

Diagnosis

Allinovi M, Trivioli G, Gaudio C, L'Imperio V, Rauf MU, Gillmore JD
Nephrol Dial Transplant 2025 Sep 29;40(10):1826-1837. doi: 10.1093/ndt/gfaf042. PMID: 40036358
Mirioglu S, Uludag O, Hurdogan O, Kumru G, Berke I, Doumas SA, Frangou E, Gul A
Curr Rheumatol Rep 2024 Jul;26(7):248-259. Epub 2024 Apr 3 doi: 10.1007/s11926-024-01147-8. PMID: 38568326Free PMC Article
Hatemi G, Seyahi E, Fresko I, Talarico R, Uçar D, Hamuryudan V
Clin Exp Rheumatol 2023 Oct;41(10):1945-1954. Epub 2023 Oct 10 doi: 10.55563/clinexprheumatol/7kdo9x. PMID: 37877363
Karam S, Haidous M, Royal V, Leung N
Kidney Int 2023 Mar;103(3):473-484. Epub 2022 Dec 9 doi: 10.1016/j.kint.2022.10.028. PMID: 36502873
Cudrici C, Deuitch N, Aksentijevich I
Int J Mol Sci 2020 May 5;21(9) doi: 10.3390/ijms21093263. PMID: 32380704Free PMC Article

Therapy

Ozen S, Sağ E, Oton T, Gül A, Sieiro Santos C, Bayraktar D, Proft FN, Lachmann HJ, Kuemmerle Deschner J, Gattorno M, Ayaz NA, Karadağ Ö, Yüce S, Kivity S, Georgin-Lavialle S, Sarkisian T, Kallinich T, Hentgen V, Prior Y, Uziel Y, Yardeni Z, Carmona L
Ann Rheum Dis 2025 Jun;84(6):899-909. Epub 2025 Apr 9 doi: 10.1016/j.ard.2025.01.028. PMID: 40234174
Hatemi G, Seyahi E, Fresko I, Talarico R, Uçar D, Hamuryudan V
Clin Exp Rheumatol 2023 Oct;41(10):1945-1954. Epub 2023 Oct 10 doi: 10.55563/clinexprheumatol/7kdo9x. PMID: 37877363
Ozdogan H, Ugurlu S
Presse Med 2019 Feb;48(1 Pt 2):e61-e76. Epub 2019 Jan 25 doi: 10.1016/j.lpm.2018.08.014. PMID: 30686512
Lachmann HJ
Best Pract Res Clin Rheumatol 2017 Aug;31(4):596-609. Epub 2018 Jan 11 doi: 10.1016/j.berh.2017.12.001. PMID: 29773275
Pinney JH, Hawkins PN
Ann Clin Biochem 2012 May;49(Pt 3):229-41. Epub 2012 Mar 8 doi: 10.1258/acb.2011.011225. PMID: 22402917

Prognosis

Tang J
R I Med J (2013) 2025 Oct 1;108(10):51-54. PMID: 41002209
Allinovi M, Trivioli G, Gaudio C, L'Imperio V, Rauf MU, Gillmore JD
Nephrol Dial Transplant 2025 Sep 29;40(10):1826-1837. doi: 10.1093/ndt/gfaf042. PMID: 40036358
Karam S, Haidous M, Royal V, Leung N
Kidney Int 2023 Mar;103(3):473-484. Epub 2022 Dec 9 doi: 10.1016/j.kint.2022.10.028. PMID: 36502873
Kozycki CT, Kodati S, Huryn L, Wang H, Warner BM, Jani P, Hammoud D, Abu-Asab MS, Jittayasothorn Y, Mattapallil MJ, Tsai WL, Ullah E, Zhou P, Tian X, Soldatos A, Moutsopoulos N, Kao-Hsieh M, Heller T, Cowen EW, Lee CR, Toro C, Kalsi S, Khavandgar Z, Baer A, Beach M, Long Priel D, Nehrebecky M, Rosenzweig S, Romeo T, Deuitch N, Brenchley L, Pelayo E, Zein W, Sen N, Yang AH, Farley G, Sweetser DA, Briere L, Yang J, de Oliveira Poswar F, Schwartz IVD, Silva Alves T, Dusser P, Koné-Paut I, Touitou I, Titah SM, van Hagen PM, van Wijck RTA, van der Spek PJ, Yano H, Benneche A, Apalset EM, Jansson RW, Caspi RR, Kuhns DB, Gadina M, Takada H, Ida H, Nishikomori R, Verrecchia E, Sangiorgi E, Manna R, Brooks BP, Sobrin L, Hufnagel RB, Beck D, Shao F, Ombrello AK, Aksentijevich I, Kastner DL; Undiagnosed Diseases Network
Ann Rheum Dis 2022 Oct;81(10):1453-1464. Epub 2022 Jul 22 doi: 10.1136/annrheumdis-2022-222629. PMID: 35868845Free PMC Article
Bansal R, Syed U, Walfish J, Aron J, Walfish A
Curr Gastroenterol Rep 2018 Mar 26;20(3):11. doi: 10.1007/s11894-018-0616-y. PMID: 29582184

Clinical prediction guides

Ayar Y, Ersoy A, Oksuz MF, Ocakoglu G, Vuruskan BA, Yildiz A, Isiktas E, Oruc A, Celikci S, Arslan I, Sahin AB, Güllülü M
Rev Bras Reumatol Engl Ed 2017 Nov-Dec;57(6):535-544. Epub 2017 Mar 23 doi: 10.1016/j.rbre.2017.02.002. PMID: 29173691
Pinney JH, Lachmann HJ
Subcell Biochem 2012;65:541-64. doi: 10.1007/978-94-007-5416-4_20. PMID: 23225016
Obici L, Merlini G
Autoimmun Rev 2012 Nov;12(1):14-7. Epub 2012 Aug 2 doi: 10.1016/j.autrev.2012.07.016. PMID: 22878269
Verine J, Mourad N, Desseaux K, Vanhille P, Noël LH, Beaufils H, Grateau G, Janin A, Droz D
Hum Pathol 2007 Dec;38(12):1798-809. Epub 2007 Aug 21 doi: 10.1016/j.humpath.2007.04.013. PMID: 17714761
Tan SY, Pepys MB, Hawkins PN
Am J Kidney Dis 1995 Aug;26(2):267-85. doi: 10.1016/0272-6386(95)90647-9. PMID: 7645531

Recent systematic reviews

De Matteis A, Bindoli S, De Benedetti F, Carmona L, Fautrel B, Mitrovic S
Ann Rheum Dis 2024 Nov 14;83(12):1748-1761. doi: 10.1136/ard-2024-225853. PMID: 39317414Free PMC Article
Karatemiz G, Esatoglu SN, Gurcan M, Ozguler Y, Yurdakul S, Hamuryudan V, Fresko I, Melikoglu M, Seyahi E, Ugurlu S, Ozdogan H, Yazici H, Hatemi G
Rheumatology (Oxford) 2022 Dec 23;62(1):9-18. doi: 10.1093/rheumatology/keac223. PMID: 35657376
Rodrigues F, Cuisset L, Cador-Rousseau B, Giurgea I, Neven B, Buob D, Quartier P, Hachulla E, Lequerré T, Cam G, Boursier G, Hervieu V, Grateau G, Georgin-Lavialle S
Rheumatology (Oxford) 2022 Nov 28;61(12):4827-4834. doi: 10.1093/rheumatology/keac145. PMID: 35262642
Brunger AF, Nienhuis HLA, Bijzet J, Hazenberg BPC
Amyloid 2020 Mar;27(1):1-12. Epub 2019 Nov 25 doi: 10.1080/13506129.2019.1693359. PMID: 31766892
Pettersson T, Konttinen YT, Maury CP
Expert Opin Pharmacother 2008 Aug;9(12):2117-28. doi: 10.1517/14656566.9.12.2117. PMID: 18671466

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