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Antiphospholipid syndrome

MedGen UID:
38834
Concept ID:
C0085278
Disease or Syndrome
Synonyms: Anti Phospholipid Antibody Syndrome; Anti Phospholipid Syndrome; Anti-Phospholipid Antibody Syndrome; Anti-Phospholipid Syndrome; Antibody Syndrome, Anti-Phospholipid; Antibody Syndrome, Antiphospholipid; Antiphospholipid Antibody Syndrome; Antiphospholipid Antibody Syndromes; Antiphospholipid Syndrome; Hughes Syndrome; Syndrome, Anti-Phospholipid; Syndrome, Anti-Phospholipid Antibody; Syndrome, Antiphospholipid; Syndrome, Antiphospholipid Antibody; Syndrome, Hughes
SNOMED CT: APS - Antiphospholipid syndrome (26843008); Anticardiolipin syndrome (26843008); APL - Antiphospholipid syndrome (26843008); Antiphospholipid syndrome (26843008)
 
Monarch Initiative: MONDO:8000010
Orphanet: ORPHA80

Definition

A disorder caused by the presence of autoantibodies directed against phospholipids, causing a hypercoaguable state, which may result in blood clots, stroke, heart attack, and in women, significant pregnancy-related complications, including miscarriage and still birth. The syndrome is often associated with other autoimmune disorders, most commonly lupus erythematosus, and infections, including syphilis and Lyme disease. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAntiphospholipid syndrome

Professional guidelines

PubMed

Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat-Chalumeau N, Cuadrado MJ, Dörner T, Ferrer-Oliveras R, Hambly K, Khamashta MA, King J, Marchiori F, Meroni PL, Mosca M, Pengo V, Raio L, Ruiz-Irastorza G, Shoenfeld Y, Stojanovich L, Svenungsson E, Wahl D, Tincani A, Ward MM
Ann Rheum Dis 2019 Oct;78(10):1296-1304. Epub 2019 May 15 doi: 10.1136/annrheumdis-2019-215213. PMID: 31092409
Garcia D, Erkan D
N Engl J Med 2018 May 24;378(21):2010-2021. doi: 10.1056/NEJMra1705454. PMID: 29791828
Chaturvedi S, McCrae KR
Blood Rev 2017 Nov;31(6):406-417. Epub 2017 Jul 30 doi: 10.1016/j.blre.2017.07.006. PMID: 28784423Free PMC Article

Recent clinical studies

Etiology

Knight JS, Branch DW, Ortel TL
BMJ 2023 Feb 27;380:e069717. doi: 10.1136/bmj-2021-069717. PMID: 36849186
Sammaritano LR
Best Pract Res Clin Rheumatol 2020 Feb;34(1):101463. Epub 2019 Dec 19 doi: 10.1016/j.berh.2019.101463. PMID: 31866276
Chaturvedi S, McCrae KR
Blood Rev 2017 Nov;31(6):406-417. Epub 2017 Jul 30 doi: 10.1016/j.blre.2017.07.006. PMID: 28784423Free PMC Article
Carmi O, Berla M, Shoenfeld Y, Levy Y
Expert Rev Hematol 2017 Apr;10(4):365-374. Epub 2017 Mar 13 doi: 10.1080/17474086.2017.1300522. PMID: 28277850
Ruiz-Irastorza G, Crowther M, Branch W, Khamashta MA
Lancet 2010 Oct 30;376(9751):1498-509. Epub 2010 Sep 6 doi: 10.1016/S0140-6736(10)60709-X. PMID: 20822807

Diagnosis

Knight JS, Kanthi Y
Semin Immunopathol 2022 May;44(3):347-362. Epub 2022 Feb 4 doi: 10.1007/s00281-022-00916-w. PMID: 35122116Free PMC Article
Xourgia E, Tektonidou MG
Curr Rheumatol Rep 2022 Jan 5;23(12):84. doi: 10.1007/s11926-021-01051-5. PMID: 34985625
Sayar Z, Moll R, Isenberg D, Cohen H
Thromb Res 2021 Feb;198:213-221. Epub 2020 Oct 15 doi: 10.1016/j.thromres.2020.10.010. PMID: 33485122Free PMC Article
Sammaritano LR
Best Pract Res Clin Rheumatol 2020 Feb;34(1):101463. Epub 2019 Dec 19 doi: 10.1016/j.berh.2019.101463. PMID: 31866276
Garcia D, Erkan D
N Engl J Med 2018 May 24;378(21):2010-2021. doi: 10.1056/NEJMra1705454. PMID: 29791828

Therapy

Knight JS, Branch DW, Ortel TL
BMJ 2023 Feb 27;380:e069717. doi: 10.1136/bmj-2021-069717. PMID: 36849186
Erton ZB, Erkan D
Curr Opin Pharmacol 2022 Aug;65:102212. Epub 2022 May 27 doi: 10.1016/j.coph.2022.102212. PMID: 35636385
Sayar Z, Moll R, Isenberg D, Cohen H
Thromb Res 2021 Feb;198:213-221. Epub 2020 Oct 15 doi: 10.1016/j.thromres.2020.10.010. PMID: 33485122Free PMC Article
Petri M
Transl Res 2020 Nov;225:70-81. Epub 2020 May 12 doi: 10.1016/j.trsl.2020.04.006. PMID: 32413497Free PMC Article
Cervera R
Thromb Res 2017 Mar;151 Suppl 1:S43-S47. doi: 10.1016/S0049-3848(17)30066-X. PMID: 28262233

Prognosis

Dabit JY, Valenzuela-Almada MO, Vallejo-Ramos S, Duarte-García A
Curr Rheumatol Rep 2022 Jan 5;23(12):85. doi: 10.1007/s11926-021-01038-2. PMID: 34985614Free PMC Article
Walter IJ, Klein Haneveld MJ, Lely AT, Bloemenkamp KWM, Limper M, Kooiman J
Autoimmun Rev 2021 Oct;20(10):102901. Epub 2021 Jul 16 doi: 10.1016/j.autrev.2021.102901. PMID: 34280554
Bala MM, Celinska-Lowenhoff M, Szot W, Padjas A, Kaczmarczyk M, Swierz MJ, Undas A
Cochrane Database Syst Rev 2020 Oct 12;10(10):CD012169. doi: 10.1002/14651858.CD012169.pub3. PMID: 33045766Free PMC Article
Pons-Estel GJ, Andreoli L, Scanzi F, Cervera R, Tincani A
J Autoimmun 2017 Jan;76:10-20. Epub 2016 Oct 21 doi: 10.1016/j.jaut.2016.10.004. PMID: 27776934
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500

Clinical prediction guides

Arachchillage DRJ, Pericleous C
Semin Thromb Hemost 2023 Apr;49(3):295-304. Epub 2023 Jan 16 doi: 10.1055/s-0042-1760333. PMID: 36646109
Pires da Rosa G, Ferreira E, Sousa-Pinto B, Rodríguez-Pintó I, Brito I, Mota A, Cervera R, Espinosa G
Front Immunol 2022;13:967178. Epub 2022 Aug 18 doi: 10.3389/fimmu.2022.967178. PMID: 36059460Free PMC Article
Barbhaiya M, Zuily S, Ahmadzadeh Y, Amigo MC, Avcin T, Bertolaccini ML, Branch DW, de Jesus G, Devreese KMJ, Frances C, Garcia D, Guillemin F, Levine SR, Levy RA, Lockshin MD, Ortel TL, Seshan SV, Tektonidou M, Wahl D, Willis R, Naden R, Costenbader K, Erkan D; New APS Classification Criteria Collaborators
Arthritis Care Res (Hoboken) 2021 Oct;73(10):1490-1501. Epub 2021 Sep 2 doi: 10.1002/acr.24520. PMID: 33253499Free PMC Article
Chaturvedi S, McCrae KR
Blood Rev 2017 Nov;31(6):406-417. Epub 2017 Jul 30 doi: 10.1016/j.blre.2017.07.006. PMID: 28784423Free PMC Article
Ruiz-Irastorza G, Crowther M, Branch W, Khamashta MA
Lancet 2010 Oct 30;376(9751):1498-509. Epub 2010 Sep 6 doi: 10.1016/S0140-6736(10)60709-X. PMID: 20822807

Recent systematic reviews

Khairani CD, Bejjani A, Piazza G, Jimenez D, Monreal M, Chatterjee S, Pengo V, Woller SC, Cortes-Hernandez J, Connors JM, Kanthi Y, Krumholz HM, Middeldorp S, Falanga A, Cushman M, Goldhaber SZ, Garcia DA, Bikdeli B
J Am Coll Cardiol 2023 Jan 3;81(1):16-30. Epub 2022 Oct 31 doi: 10.1016/j.jacc.2022.10.008. PMID: 36328154Free PMC Article
Walter IJ, Klein Haneveld MJ, Lely AT, Bloemenkamp KWM, Limper M, Kooiman J
Autoimmun Rev 2021 Oct;20(10):102901. Epub 2021 Jul 16 doi: 10.1016/j.autrev.2021.102901. PMID: 34280554
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Andreoli L, Bertsias GK, Agmon-Levin N, Brown S, Cervera R, Costedoat-Chalumeau N, Doria A, Fischer-Betz R, Forger F, Moraes-Fontes MF, Khamashta M, King J, Lojacono A, Marchiori F, Meroni PL, Mosca M, Motta M, Ostensen M, Pamfil C, Raio L, Schneider M, Svenungsson E, Tektonidou M, Yavuz S, Boumpas D, Tincani A
Ann Rheum Dis 2017 Mar;76(3):476-485. Epub 2016 Jul 25 doi: 10.1136/annrheumdis-2016-209770. PMID: 27457513Free PMC Article
Tunnicliffe DJ, Singh-Grewal D, Kim S, Craig JC, Tong A
Arthritis Care Res (Hoboken) 2015 Oct;67(10):1440-52. doi: 10.1002/acr.22591. PMID: 25778500

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