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Lupus anticoagulant disorder

MedGen UID:
551915
•Concept ID:
C0311370
•
Disease or Syndrome
Synonym: Lupus anticoagulant
SNOMED CT: Lupus anticoagulant (19267009); Lupus anticoagulant disorder (19267009); Lupus anticoagulant inhibitor syndrome (19267009); SLE inhibitor syndrome (19267009)

Definition

A hypercoaguable state that results from the presence of the immunoglobulin known as Lupus Anticoagulant. The antibody interacts with cell membrane phospholipids, causing increased aggregation and adhesion of platelets, which causes increased clot formation. Though the majority of patients who test positive for lupus anticoagulant do not have lupus, those individuals afflicted with lupus have a higher probability of developing the antibody. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLupus anticoagulant disorder

Professional guidelines

PubMed

2023 ACR/EULAR antiphospholipid syndrome classification criteria.
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
The 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria.
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
Diagnosis and management of the antiphospholipid syndrome.
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

Antiphospholipid syndrome.
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
Management of maternal antiphospholipid syndrome.
Fernandes MA, Gerardi MC, Andreoli L, Tincani A
Clin Exp Rheumatol 2020 Jan-Feb;38(1):149-156. Epub 2019 Jun 28 PMID: 31287415
Laboratory Diagnostics in Thrombophilia.
Linnemann B, Hart C
Hamostaseologie 2019 Feb;39(1):49-61. Epub 2019 Jan 31 doi: 10.1055/s-0039-1677840. PMID: 30703819
Lupus anticoagulant.
Creagh MD, Greaves M
Blood Rev 1991 Sep;5(3):162-7. doi: 10.1016/0268-960x(91)90033-9. PMID: 1777749
Antiphospholipid antibodies.
Levine SR, Welch KM
Ann Neurol 1989 Sep;26(3):386-9. doi: 10.1002/ana.410260312. PMID: 2508536

Diagnosis

Survey on antiphospholipid syndrome diagnosis and antithrombotic treatment in patients with ischemic stroke, other brain ischemic injury, or arterial thromboembolism in other sites: communication from ISTH SSC Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibodies.
Cohen H, Werring DJ, Chandratheva A, Mittal P, Devreese KMJ, Isenberg DA; ISTH SSC Lupus Anticoagulant/Antiphospholipid Antibodies Subcommittee Study Group
J Thromb Haemost 2023 Oct;21(10):2963-2976. Epub 2023 Jun 28 doi: 10.1016/j.jtha.2023.06.020. PMID: 37391096
The history of antiphospholipid syndrome.
Tincani A, Fontana G, Mackworth-Young C
Reumatismo 2023 Mar 21;74(4) doi: 10.4081/reumatismo.2022.1556. PMID: 36942979
Seronegative antiphospholipid syndrome: refining the value of "non-criteria" antibodies for diagnosis and clinical management.
Pignatelli P, Ettorre E, Menichelli D, Pani A, Violi F, Pastori D
Haematologica 2020 Mar;105(3):562-572. Epub 2020 Jan 30 doi: 10.3324/haematol.2019.221945. PMID: 32001534Free PMC Article
Antiphospholipid syndrome.
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
Laboratory Diagnostics in Thrombophilia.
Linnemann B, Hart C
Hamostaseologie 2019 Feb;39(1):49-61. Epub 2019 Jan 31 doi: 10.1055/s-0039-1677840. PMID: 30703819

Therapy

Levothyroxine in euthyroid thyroid peroxidase antibody positive women with recurrent pregnancy loss (T4LIFE trial): a multicentre, randomised, double-blind, placebo-controlled, phase 3 trial.
van Dijk MM, Vissenberg R, Fliers E, van der Post JAM, van der Hoorn MP, de Weerd S, Kuchenbecker WK, Hoek A, Sikkema JM, Verhoeve HR, Broeze KA, de Koning CH, Verpoest W, Christiansen OB, Koks C, de Bruin JP, Papatsonis DNM, Torrance H, van Wely M, Bisschop PH, Goddijn M
Lancet Diabetes Endocrinol 2022 May;10(5):322-329. Epub 2022 Mar 14 doi: 10.1016/S2213-8587(22)00045-6. PMID: 35298917
Laboratory Diagnostics in Thrombophilia.
Linnemann B, Hart C
Hamostaseologie 2019 Feb;39(1):49-61. Epub 2019 Jan 31 doi: 10.1055/s-0039-1677840. PMID: 30703819
Management of antiphospholipid syndrome.
Uthman I, Noureldine MHA, Ruiz-Irastorza G, Khamashta M
Ann Rheum Dis 2019 Feb;78(2):155-161. Epub 2018 Oct 3 doi: 10.1136/annrheumdis-2018-213846. PMID: 30282668
Antiphospholipid syndrome.
Schreiber K, Sciascia S, de Groot PG, Devreese K, Jacobsen S, Ruiz-Irastorza G, Salmon JE, Shoenfeld Y, Shovman O, Hunt BJ
Nat Rev Dis Primers 2018 Jan 11;4:17103. doi: 10.1038/nrdp.2017.103. PMID: 29321641
Diagnosis and management of the antiphospholipid syndrome.
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

Prognosis

Diagnosis and management of the antiphospholipid syndrome.
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
Lupus Nephritis and Pregnancy: Concerns and Management.
Lightstone L, Hladunewich MA
Semin Nephrol 2017 Jul;37(4):347-353. doi: 10.1016/j.semnephrol.2017.05.006. PMID: 28711073
Diagnosis and management of catastrophic antiphospholipid syndrome.
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
Predictors of Pregnancy Outcomes in Patients With Lupus: A Cohort Study.
Buyon JP, Kim MY, Guerra MM, Laskin CA, Petri M, Lockshin MD, Sammaritano L, Branch DW, Porter TF, Sawitzke A, Merrill JT, Stephenson MD, Cohn E, Garabet L, Salmon JE
Ann Intern Med 2015 Aug 4;163(3):153-63. doi: 10.7326/M14-2235. PMID: 26098843Free PMC Article
Antiphospholipid syndrome.
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

Clinical prediction guides

The 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria.
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
Positive antiphospholipid antibodies: observation or treatment?
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
Diagnosis and management of the antiphospholipid syndrome.
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
Lupus Nephritis and Pregnancy: Concerns and Management.
Lightstone L, Hladunewich MA
Semin Nephrol 2017 Jul;37(4):347-353. doi: 10.1016/j.semnephrol.2017.05.006. PMID: 28711073
Antiphospholipid syndrome.
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

Lupus anticoagulant-hypoprothrombinemia syndrome in children: Three case reports and systematic review of the literature.
Sun YH, Nie WJ, Tian DD, Ye Q
Lupus 2023 Sep;32(10):1211-1221. Epub 2023 Jul 22 doi: 10.1177/09612033231191153. PMID: 37480550
Antiphospholipid antibodies and lower extremity peripheral artery disease: A systematic review and meta-analysis.
Merashli M, Bucci T, Pastori D, Pignatelli P, Marottoli V, Arcaro A, Gentile F, Ames PR
Semin Arthritis Rheum 2020 Dec;50(6):1291-1298. Epub 2020 Aug 30 doi: 10.1016/j.semarthrit.2020.08.012. PMID: 33065424
Antiphospholipid Antibodies and Autoimmune Haemolytic Anaemia: A Systematic Review and Meta-Analysis.
Ames PRJ, Merashli M, Bucci T, Pastori D, Pignatelli P, Arcaro A, Gentile F
Int J Mol Sci 2020 Jun 9;21(11) doi: 10.3390/ijms21114120. PMID: 32527000Free PMC Article
Comorbid association of antiphospholipid antibodies and migraine: A systematic review and meta-analysis.
Islam MA, Alam F, Wong KK
Autoimmun Rev 2017 May;16(5):512-522. Epub 2017 Mar 7 doi: 10.1016/j.autrev.2017.03.005. PMID: 28279839
Antiphospholipid antibody and risk of retinal vein occlusion: a systematic review and meta-analysis.
Zhu W, Wu Y, Xu M, Wang JY, Meng YF, Gu Z, Lu J
PLoS One 2014;10(4):e0122814. Epub 2015 Apr 28 doi: 10.1371/journal.pone.0122814. PMID: 25918850Free PMC Article

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