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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 protocol for the diagnosis and management of familial Mediterranean fever.
Georgin-Lavialle S, Savey L, Cuisset L, Boursier G, Boffa JJ, Delplanque M, Bourguiba R, Monfort JB, Touitou I, Grateau G; Collaborators, Kone-Paut I, Hentgen V
Rev Med Interne 2023 Nov;44(11):602-616. Epub 2023 Oct 29 doi: 10.1016/j.revmed.2023.10.441. PMID: 37903671
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
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

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
Renal sarcoidosis: approach to diagnosis and management.
Bergner R, Löffler C
Curr Opin Pulm Med 2018 Sep;24(5):513-520. doi: 10.1097/MCP.0000000000000504. PMID: 29965860
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
Prognostication of survival and progression to dialysis in AA amyloidosis.
Palladini G, Riva E, Basset M, Russo F, Milani P, Pasquinucci E, Foli A, Lavatelli F, Nuvolone M, Casarini S, Obici L, Merlini G
Amyloid 2017 Mar;24(sup1):136-137. doi: 10.1080/13506129.2017.1289917. PMID: 28434292

Diagnosis

AA Amyloidosis: A Contemporary View.
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
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
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
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

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
The systemic autoinflammatory disorders for dermatologists. Part 2: disease examples.
Oldham J, Lachmann HJ
Clin Exp Dermatol 2020 Dec;45(8):967-973. Epub 2020 Sep 3 doi: 10.1111/ced.14251. PMID: 32882069
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
Setting up TRAPS.
Pettersson T, Kantonen J, Matikainen S, Repo H
Ann Med 2012 Mar;44(2):109-18. Epub 2011 Feb 1 doi: 10.3109/07853890.2010.548399. PMID: 21284532

Prognosis

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
Systemic AA amyloidosis.
Pinney JH, Lachmann HJ
Subcell Biochem 2012;65:541-64. doi: 10.1007/978-94-007-5416-4_20. PMID: 23225016
AA amyloidosis and AL amyloidosis.
Isobe T
Intern Med 1993 Dec;32(12):919-20. doi: 10.2169/internalmedicine.32.919. PMID: 8204969

Clinical prediction guides

Development and Validation of Staging Systems for AA Amyloidosis.
Basset M, Schönland SO, Obici L, Günther J, Riva E, Dittrich T, Milani P, Ferretti VV, Pasquinucci E, Foli A, Kimmich C, Nanci M, Bellofiore C, Benigna F, Beimler J, Benvenuti P, Fabris F, Mussinelli R, Nuvolone M, Klersy C, Albertini R, Merlini G, Hegenbart U, Palladini G, Blank N
J Am Soc Nephrol 2024 Jun 1;35(6):782-794. Epub 2024 Mar 21 doi: 10.1681/ASN.0000000000000339. PMID: 38512269Free 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
Systemic AA amyloidosis.
Pinney JH, Lachmann HJ
Subcell Biochem 2012;65:541-64. doi: 10.1007/978-94-007-5416-4_20. PMID: 23225016
Familial Mediterranean fever and mesangial proliferative glomerulonephritis: report of a case and review of the literature.
Cagdas DN, Gucer S, Kale G, Duzova A, Ozen S
Pediatr Nephrol 2005 Sep;20(9):1352-4. Epub 2005 Jun 22 doi: 10.1007/s00467-005-1991-9. PMID: 15971069
Treatment of amyloidosis.
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

AA-type amyloidosis associated with lymphoma: a study of 19 cases including 5 new French cases and a systematic literature review.
Collet A, Attias P, Lacou M, Fieschi C, Moktefi A, Sannier A, Grateau G, Buob D, Georgin-Lavialle S
Leuk Lymphoma 2023 Nov-Dec;64(11):1857-1863. Epub 2023 Jul 26 doi: 10.1080/10428194.2023.2239971. PMID: 37493601
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
AA amyloidosis secondary to adult onset Still's disease: About 19 cases.
Delplanque M, Pouchot J, Ducharme-Bénard S, Fautrel BJ, Benyamine A, Daniel L, Gendre T, Grateau G, Georgin-Lavialle S
Semin Arthritis Rheum 2020 Feb;50(1):156-165. Epub 2019 Aug 10 doi: 10.1016/j.semarthrit.2019.08.005. PMID: 31488308

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