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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

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
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
Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, Derksen RH, DE Groot PG, Koike T, Meroni PL, Reber G, Shoenfeld Y, Tincani A, Vlachoyiannopoulos PG, Krilis SA
J Thromb Haemost 2006 Feb;4(2):295-306. doi: 10.1111/j.1538-7836.2006.01753.x. PMID: 16420554

Diagnosis

Knight JS, Branch DW, Ortel TL
BMJ 2023 Feb 27;380:e069717. doi: 10.1136/bmj-2021-069717. PMID: 36849186
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
Meroni PL, Tsokos GC
Front Immunol 2019;10:199. Epub 2019 Feb 25 doi: 10.3389/fimmu.2019.00199. PMID: 30858846Free PMC Article
Garcia D, Erkan D
N Engl J Med 2018 May 24;378(21):2010-2021. doi: 10.1056/NEJMra1705454. PMID: 29791828

Therapy

Bitsadze V, Yakubova F, Khizroeva J, Lazarchuk A, Salnikova P, Vorobev A, Tretyakova M, Degtyareva N, Grigoreva K, Gashimova N, Kvaratskheliia M, Makatsariya N, Kudryavtseva E, Tomlenova A, Gris JC, Elalamy I, Ay C, Makatsariya A
Int J Mol Sci 2024 Jan 4;25(1) doi: 10.3390/ijms25010668. PMID: 38203837Free PMC Article
Knight JS, Branch DW, Ortel TL
BMJ 2023 Feb 27;380:e069717. doi: 10.1136/bmj-2021-069717. PMID: 36849186
Mittal P, Quattrocchi G, Tohidi-Esfahani I, Sayar Z, Chandratheva A, Cohen H
Int J Stroke 2023 Apr;18(4):383-391. Epub 2023 Jan 25 doi: 10.1177/17474930221150349. PMID: 36571450
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

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
Vreede AP, Bockenstedt PL, Knight JS
Curr Opin Rheumatol 2017 Sep;29(5):458-466. doi: 10.1097/BOR.0000000000000410. PMID: 28538012Free PMC Article
Cohen H, Hunt BJ, Efthymiou M, Arachchillage DR, Mackie IJ, Clawson S, Sylvestre Y, Machin SJ, Bertolaccini ML, Ruiz-Castellano M, Muirhead N, Doré CJ, Khamashta M, Isenberg DA; RAPS trial investigators
Lancet Haematol 2016 Sep;3(9):e426-36. doi: 10.1016/S2352-3026(16)30079-5. PMID: 27570089Free PMC Article
Silver RM
Obstet Gynecol 2007 Jan;109(1):153-67. doi: 10.1097/01.AOG.0000248537.89739.96. PMID: 17197601

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
Grygiel-Górniak B, Mazurkiewicz Ł
J Thromb Thrombolysis 2023 Aug;56(2):301-314. Epub 2023 Jun 1 doi: 10.1007/s11239-023-02834-6. PMID: 37264223Free PMC Article
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
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
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

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
Asmis L, Hellstern P
Clin Lab 2023 Apr 1;69(4) doi: 10.7754/Clin.Lab.2022.220817. PMID: 37057948
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
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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