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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

Fanouriakis A, Kostopoulou M, Andersen J, Aringer M, Arnaud L, Bae SC, Boletis J, Bruce IN, Cervera R, Doria A, Dörner T, Furie RA, Gladman DD, Houssiau FA, Inês LS, Jayne D, Kouloumas M, Kovács L, Mok CC, Morand EF, Moroni G, Mosca M, Mucke J, Mukhtyar CB, Nagy G, Navarra S, Parodis I, Pego-Reigosa JM, Petri M, Pons-Estel BA, Schneider M, Smolen JS, Svenungsson E, Tanaka Y, Tektonidou MG, Teng YO, Tincani A, Vital EM, van Vollenhoven RF, Wincup C, Bertsias G, Boumpas DT
Ann Rheum Dis 2024 Jan 2;83(1):15-29. doi: 10.1136/ard-2023-224762. PMID: 37827694
Barbhaiya M, Zuily S, Naden R, Hendry A, Manneville F, Amigo MC, Amoura Z, Andrade D, Andreoli L, Artim-Esen B, Atsumi T, Avcin T, Belmont HM, Bertolaccini ML, Branch DW, Carvalheiras G, Casini A, Cervera R, Cohen H, Costedoat-Chalumeau N, Crowther M, de Jesus G, Delluc A, Desai S, De Sancho M, Devreese KM, Diz-Kucukkaya R, Duarte-Garcia A, Frances C, Garcia D, Gris JC, Jordan N, Leaf RK, Kello N, Knight JS, Laskin C, Lee AI, Legault K, Levine SR, Levy RA, Limper M, Lockshin MD, Mayer-Pickel K, Musial J, Meroni PL, Orsolini G, Ortel TL, Pengo V, Petri M, Pons-Estel G, Gomez-Puerta JA, Raimboug Q, Roubey R, Sanna G, Seshan SV, Sciascia S, Tektonidou MG, Tincani A, Wahl D, Willis R, Yelnik C, Zuily C, Guillemin F, Costenbader K, Erkan D; ACR/EULAR APS Classification Criteria Collaborators
Arthritis Rheumatol 2023 Oct;75(10):1687-1702. Epub 2023 Aug 28 doi: 10.1002/art.42624. PMID: 37635643
Knight JS, Branch DW, Ortel TL
BMJ 2023 Feb 27;380:e069717. doi: 10.1136/bmj-2021-069717. PMID: 36849186

Recent clinical studies

Etiology

Andreoli L, Regola F, Caproli A, Crisafulli F, Fredi M, Lazzaroni MG, Nalli C, Piantoni S, Zatti S, Franceschini F, Tincani A
Rheumatology (Oxford) 2024 Feb 6;63(SI):SI86-SI95. doi: 10.1093/rheumatology/kead537. PMID: 38320595
Knight JS, Branch DW, Ortel TL
BMJ 2023 Feb 27;380:e069717. doi: 10.1136/bmj-2021-069717. PMID: 36849186
López-Benjume B, Rodríguez-Pintó I, Amigo MC, Erkan D, Shoenfeld Y, Cervera R, Espinosa G; on behalf the CAPS Registry Project Group/European Forum on Antiphospholipid Antibodies
Autoimmun Rev 2022 Apr;21(4):103055. Epub 2022 Jan 24 doi: 10.1016/j.autrev.2022.103055. PMID: 35085802
Leal Rato M, Bandeira M, Romão VC, Aguiar de Sousa D
Curr Neurol Neurosci Rep 2021 Jun 14;21(8):41. doi: 10.1007/s11910-021-01124-z. PMID: 34125304Free PMC Article
Houghton DE, Moll S
Vasc Med 2017 Dec;22(6):545-550. Epub 2017 Nov 5 doi: 10.1177/1358863X17737374. PMID: 29103369

Diagnosis

Knight JS, Branch DW, Ortel TL
BMJ 2023 Feb 27;380:e069717. doi: 10.1136/bmj-2021-069717. PMID: 36849186
Leal Rato M, Bandeira M, Romão VC, Aguiar de Sousa D
Curr Neurol Neurosci Rep 2021 Jun 14;21(8):41. doi: 10.1007/s11910-021-01124-z. PMID: 34125304Free PMC Article
Serrano A, Cervera R, Gris JC
Front Immunol 2020;11:1993. Epub 2020 Aug 28 doi: 10.3389/fimmu.2020.01993. PMID: 32983139Free PMC Article
Garcia D, Erkan D
N Engl J Med 2018 May 24;378(21):2010-2021. doi: 10.1056/NEJMra1705454. PMID: 29791828
Aguiar CL, Soybilgic A, Avcin T, Myones BL
Curr Rheumatol Rep 2015 Apr;17(4):27. doi: 10.1007/s11926-015-0504-5. PMID: 25854492

Therapy

Girón-Ortega JA, Girón-González JA
Med Clin (Barc) 2023 Jul 21;161(2):65-77. Epub 2023 Apr 25 doi: 10.1016/j.medcli.2023.03.011. PMID: 37105842
Knight JS, Branch DW, Ortel TL
BMJ 2023 Feb 27;380:e069717. doi: 10.1136/bmj-2021-069717. PMID: 36849186
Rahman A
Clin Rheumatol 2020 Jul;39(7):2111-2114. Epub 2020 May 26 doi: 10.1007/s10067-020-05183-4. PMID: 32458245
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
Ordi-Ros J, Sáez-Comet L, Pérez-Conesa M, Vidal X, Riera-Mestre A, Castro-Salomó A, Cuquet-Pedragosa J, Ortiz-Santamaria V, Mauri-Plana M, Solé C, Cortés-Hernández J
Ann Intern Med 2019 Nov 19;171(10):685-694. Epub 2019 Oct 15 doi: 10.7326/M19-0291. PMID: 31610549

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
Skeith L, Blondon M, Ní Áinle F
Hamostaseologie 2020 Aug;40(3):356-363. Epub 2020 Jul 29 doi: 10.1055/a-1184-8388. PMID: 32726830
Miesbach W
Clin Rev Allergy Immunol 2009 Jun;36(2-3):91-7. doi: 10.1007/s12016-008-8101-2. PMID: 19048416
Duckitt K, Qureshi A
Am Fam Physician 2008 Oct 15;78(8):977-8. PMID: 18953976
Davie CA, O'Brien P
J Neurol Neurosurg Psychiatry 2008 Mar;79(3):240-5. Epub 2007 Nov 6 doi: 10.1136/jnnp.2007.116939. PMID: 17986502

Clinical prediction guides

Barbhaiya M, Zuily S, Naden R, Hendry A, Manneville F, Amigo MC, Amoura Z, Andrade D, Andreoli L, Artim-Esen B, Atsumi T, Avcin T, Belmont HM, Bertolaccini ML, Branch DW, Carvalheiras G, Casini A, Cervera R, Cohen H, Costedoat-Chalumeau N, Crowther M, de Jesús G, Delluc A, Desai S, Sancho M, Devreese KM, Diz-Kucukkaya R, Duarte-García A, Frances C, Garcia D, Gris JC, Jordan N, Leaf RK, Kello N, Knight JS, Laskin C, Lee AI, Legault K, Levine SR, Levy RA, Limper M, Lockshin MD, Mayer-Pickel K, Musial J, Meroni PL, Orsolini G, Ortel TL, Pengo V, Petri M, Pons-Estel G, Gomez-Puerta JA, Raimboug Q, Roubey R, Sanna G, Seshan SV, Sciascia S, Tektonidou MG, Tincani A, Wahl D, Willis R, Yelnik C, Zuily C, Guillemin F, Costenbader K, Erkan D; ACR/EULAR APS Classification Criteria Collaborators
Ann Rheum Dis 2023 Oct;82(10):1258-1270. Epub 2023 Aug 28 doi: 10.1136/ard-2023-224609. PMID: 37640450
Devreese KMJ, Ortel TL, Pengo V, de Laat B; Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibodies
J Thromb Haemost 2018 Apr;16(4):809-813. Epub 2018 Mar 13 doi: 10.1111/jth.13976. PMID: 29532986
Houghton DE, Moll S
Vasc Med 2017 Dec;22(6):545-550. Epub 2017 Nov 5 doi: 10.1177/1358863X17737374. PMID: 29103369
Espinosa G, Rodríguez-Pintó I, Cervera R
Panminerva Med 2017 Sep;59(3):254-268. Epub 2017 May 8 doi: 10.23736/S0031-0808.17.03324-9. PMID: 28488841
Greinacher A, Althaus K, Krauel K, Selleng S
Hamostaseologie 2010 Jan;30(1):17-8, 20-8. PMID: 20162249

Recent systematic reviews

Cheng CY, Baritussio A, Giordani AS, Iliceto S, Marcolongo R, Caforio ALP
Autoimmun Rev 2022 Apr;21(4):103037. Epub 2022 Jan 5 doi: 10.1016/j.autrev.2022.103037. PMID: 34995763
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
Abdel-Wahab N, Lopez-Olivo MA, Pinto-Patarroyo GP, Suarez-Almazor ME
Lupus 2016 Dec;25(14):1520-1531. Epub 2016 Apr 7 doi: 10.1177/0961203316640912. PMID: 27060064Free PMC Article

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