U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

AA amyloidosis

MedGen UID:
782429
•Concept ID:
C3536715
•
Disease or Syndrome
Synonyms: Amyloid A amyloidosis; 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.
See: This record
Not genetically inherited (Orphanet)
 
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
Familial periodic fever (FPF) is an autoinflammatory disorder characterized by recurrent fever with localized myalgia and painful erythema. Febrile attacks may last 1 or 2 days but often last longer than 1 week. Arthralgia of large joints, abdominal pain, conjunctivitis, and periorbital edema are common features. During attacks, painless cutaneous lesions may develop on the trunk or extremities and may migrate distally (review by Drenth and van der Meer, 2001).
See: Condition Record
TNF receptor-associated periodic fever syndrome (TRAPS)

Professional guidelines

PubMed

French practical guidelines for the diagnosis and management of AA amyloidosis.
Georgin-Lavialle S, Savey L, Buob D, Bastard JP, Fellahi S, Karras A, Boffa JJ, Grateau G; Collaborators, Audard V, Bridoux F, Damade R, Deshayes S, Giurgea I, Granel B, Hachulla E, Hot A, Jaccard A, Knebelmann B, Marciano S, Pelcot F, Sarrabay G, Boursier G, Sellam J, Terre A, Bourguiba R
Rev Med Interne 2023 Feb;44(2):62-71. Epub 2023 Jan 23 doi: 10.1016/j.revmed.2022.12.004. PMID: 36759076
Renal Amyloidosis: Presentation, Diagnosis, and Management.
Gurung R, Li T
Am J Med 2022 Apr;135 Suppl 1:S38-S43. Epub 2022 Jan 24 doi: 10.1016/j.amjmed.2022.01.003. PMID: 35085515
AL amyloidosis: advances in diagnostics and treatment.
Ryšavá R
Nephrol Dial Transplant 2019 Sep 1;34(9):1460-1466. doi: 10.1093/ndt/gfy291. PMID: 30299492

Recent clinical studies

Etiology

Infections and AA amyloidosis: An overview.
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
Familial Mediterranean Fever.
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
AL amyloidosis: advances in diagnostics and treatment.
Ryšavá R
Nephrol Dial Transplant 2019 Sep 1;34(9):1460-1466. doi: 10.1093/ndt/gfy291. PMID: 30299492
AA amyloidosis - Benefits and prospects of IL-6 inhibitors.
Okuda Y
Mod Rheumatol 2019 Mar;29(2):268-274. Epub 2018 Oct 2 doi: 10.1080/14397595.2018.1515145. PMID: 30132351
Small Bowel Amyloidosis.
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

Behçet's syndrome: one year in review 2023.
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
Renal AA amyloidosis: presentation, diagnosis, and current therapeutic options: a review.
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
AL amyloidosis: advances in diagnostics and treatment.
Ryšavá R
Nephrol Dial Transplant 2019 Sep 1;34(9):1460-1466. doi: 10.1093/ndt/gfy291. PMID: 30299492
Secondary, AA, Amyloidosis.
Papa R, Lachmann HJ
Rheum Dis Clin North Am 2018 Nov;44(4):585-603. Epub 2018 Sep 7 doi: 10.1016/j.rdc.2018.06.004. PMID: 30274625
Periodic fever syndromes.
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

Therapy

Behçet's syndrome: one year in review 2023.
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
French practical guidelines for the diagnosis and management of AA amyloidosis.
Georgin-Lavialle S, Savey L, Buob D, Bastard JP, Fellahi S, Karras A, Boffa JJ, Grateau G; Collaborators, Audard V, Bridoux F, Damade R, Deshayes S, Giurgea I, Granel B, Hachulla E, Hot A, Jaccard A, Knebelmann B, Marciano S, Pelcot F, Sarrabay G, Boursier G, Sellam J, Terre A, Bourguiba R
Rev Med Interne 2023 Feb;44(2):62-71. Epub 2023 Jan 23 doi: 10.1016/j.revmed.2022.12.004. PMID: 36759076
Familial Mediterranean Fever.
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
Periodic fever syndromes.
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
Amyloidosis.
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

The spectrum of pulmonary amyloidosis.
Riehani A, Soubani AO
Respir Med 2023 Nov;218:107407. Epub 2023 Sep 9 doi: 10.1016/j.rmed.2023.107407. PMID: 37696313
Renal AA amyloidosis: presentation, diagnosis, and current therapeutic options: a review.
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
AL amyloidosis: advances in diagnostics and treatment.
Ryšavá R
Nephrol Dial Transplant 2019 Sep 1;34(9):1460-1466. doi: 10.1093/ndt/gfy291. PMID: 30299492
Small Bowel Amyloidosis.
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
Amyloidosis.
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

Clinical prediction guides

AA-amyloidosis in cats (Felis catus) housed in shelters.
Ferri F, Ferro S, Porporato F, Callegari C, Guglielmetti C, Mazza M, Ferrero M, Crinò C, Gallo E, Drigo M, Coppola LM, Gerardi G, Schulte TP, Ricagno S, Vogel M, Storni F, Bachmann MF, Vogt AC, Caminito S, Mazzini G, Lavatelli F, Palladini G, Merlini G, Zini E
PLoS One 2023;18(3):e0281822. Epub 2023 Mar 29 doi: 10.1371/journal.pone.0281822. PMID: 36989207Free PMC Article
The Clinical Features and Outcomes of Renal Amyloidosis in Tunisia.
Ayed A, Salem MB, Letaief A, Salah MB, Handous I, Hamouda M, Aloui S, Skhiri H
Saudi J Kidney Dis Transpl 2022 May-Jun;33(3):432-439. doi: 10.4103/1319-2442.385967. PMID: 37843145
AA renal amyloidosis: Clinical observations over 20 years.
Khellaf G, Benziane A, Kaci L, Benabadji M
Clin Nephrol 2022 Mar;97(3):167-172. doi: 10.5414/CN110577. PMID: 34889732
Acquired and inherited amyloidosis: Knowledge driving patients' care.
Obici L, Adams D
J Peripher Nerv Syst 2020 Jun;25(2):85-101. doi: 10.1111/jns.12381. PMID: 32378274
Systemic AA amyloidosis.
Pinney JH, Lachmann HJ
Subcell Biochem 2012;65:541-64. doi: 10.1007/978-94-007-5416-4_20. PMID: 23225016

Recent systematic reviews

Frequency of AA amyloidosis has decreased in Behçet's syndrome: a retrospective study with long-term follow-up and a systematic review.
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
AA amyloidosis complicating cryopyrin-associated periodic syndrome: a study of 86 cases including 23 French patients and systematic review.
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
Causes of AA amyloidosis: a systematic review.
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
Evidence-based recommendations for genetic diagnosis of familial Mediterranean fever.
Giancane G, Ter Haar NM, Wulffraat N, Vastert SJ, Barron K, Hentgen V, Kallinich T, Ozdogan H, Anton J, Brogan P, Cantarini L, Frenkel J, Galeotti C, Gattorno M, Grateau G, Hofer M, Kone-Paut I, Kuemmerle-Deschner J, Lachmann HJ, Simon A, Demirkaya E, Feldman B, Uziel Y, Ozen S
Ann Rheum Dis 2015 Apr;74(4):635-41. Epub 2015 Jan 27 doi: 10.1136/annrheumdis-2014-206844. PMID: 25628446
Treatment strategies for amyloid A amyloidosis.
Pettersson T, Konttinen YT, Maury CP
Expert Opin Pharmacother 2008 Aug;9(12):2117-28. doi: 10.1517/14656566.9.12.2117. PMID: 18671466

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • GTR
      Related information in GTR
    • GTR(Clinical)
      Clinical tests in GTR
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...