関: antiphospholipid protein antibodies

WordNet

medicine that prevents or retards the clotting of blood (同)anticoagulant medication, decoagulant
any of several forms of ulcerative skin disease
a constellation in the southern hemisphere near Centaurus

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(特に血液の)凝固防止剤(物質) / 血液の凝固を防止する
狼瘡(ろうそう)(皮膚結核の一種)

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/01/29 14:13:41」(JST)

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Lupus anticoagulant (also known as lupus antibody, LA, LAC, or lupus inhibitors) is an immunoglobulin^[1] that binds to phospholipids and proteins associated with the cell membrane. Lupus anticoagulant is a misnomer, as it is actually a prothrombotic agent. Lupus anticoagulant antibodies in living systems cause an increase in inappropriate blood clotting. The name derives from their properties in vitro, since in laboratory tests, these antibodies increase aPTT. Investigators speculate that the antibodies interfere with phospholipids used to induce in vitro coagulation. In vivo, the antibodies are thought to interact with platelet membrane phospholipids, increasing adhesion and aggregation of platelets, which accounts for the in vivo prothrombotic characteristics.

The condition was first described by hematologist C. Lockard Conley.^[2]^[3]

Terminology

Both words in the term "lupus anticoagulant" can be misleading:

Most patients with a lupus anticoagulant do not actually have lupus erythematosus, and only a small proportion will proceed to develop this disease (which causes joint pains, skin problems and renal failure, amongst other complications). Patients with lupus erythematosus are more likely to develop a lupus anticoagulant than the general population.

The term "anticoagulant" accurately describes its function in vitro. However in vivo, it functions as a pro-coagulant.^[4]

Workup

The presence of prolonged clotting times on a routine plasma test often triggers functional testing of the blood clotting function, as well as serological testing to identify common autoantibodies such as antiphospholipid antibodies. These antibodies tend to delay in-vitro coagulation in phospholipid-dependent laboratory tests such as the partial thromboplastin time.

The initial workup of a prolonged PTT is a mixing test whereby the patient's plasma is mixed with normal pooled plasma and the clotting is re-assessed. If a clotting inhibitor such as a lupus anticoagulant is present, the inhibitor will interact with the normal pooled plasma and the clotting time will remain abnormal. However, if the clotting time of the mixed plasma corrects towards normal, the diagnosis of an inhibitor such as the lupus anticoagulant is excluded; the diagnosis is a deficient clotting factor that is replenished by the normal plasma.

If the mixing test indicates an inhibitor, diagnosis of a lupus anticoagulant is then confirmed with phospholipid-sensitive functional clotting testing, such as the dilute Russell's viper venom time, or the Kaolin clotting time. Excess phospholipid will eventually correct the prolongation of these prolonged clotting tests (conceptually known as "phospholipid neutralization" in the clinical coagulation laboratory), confirming the diagnosis of a lupus anticoagulant.

Treatment

Treatment for a lupus anticoagulant is usually undertaken in the context of documented thrombosis, such as extremity phlebitis or dural sinus vein thrombosis. Patients with a well-documented (i.e., present at least twice) lupus anticoagulant and a history of thrombosis should be considered candidates for indefinite treatment with anticoagulants. Patients with no history of thrombosis and a lupus anticoagulant should probably be observed. Current evidence suggests that the risk of recurrent thrombosis in patients with an antiphospholipid antibody is enhanced whether that antibody is measured on serological testing or functional testing. The Sapporo criteria specify that both serological and functional tests must be positive to diagnose the antiphospholipid antibody syndrome.^[5]

Miscarriages may be more prevalent in patients with a lupus anticoagulant. Some of these miscarriages may potentially be prevented with the administration of aspirin and unfractionated heparin. The Cochrane Database of Systematic Reviews provide a deeper understanding on the subject.^[6]

Thrombosis is treated with anticoagulants (LMWHs and warfarin).^[7]

References

^ Antonia Joussen; T.W. Gardner; B. Kirchhof (23 October 2007). Retinal Vascular Disease. Springer. pp. 430–. ISBN 978-3-540-29541-9. Retrieved 29 June 2010.
^ Conley, C. Lockard (1952). "A hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erythematosus". Journal of Clinical Investigation 31: 621–622. doi:10.1172/JCI102648.
^ "Lock Conley looks back and blushes". Hopkins Medicine. Spring–Summer 2006. Retrieved 5 December 2013.
^ "wustl.edu". Retrieved 2009-02-17.
^ Viard JP, Amoura Z, Bach JF (1991). "[Anti-beta 2 glycoprotein I antibodies in systemic lupus erythematosus: a marker of thrombosis associated with a circulating anticoagulant]". C. R. Acad. Sci. III, Sci. Vie (in French) 313 (13): 607–12. PMID 1782567.
^ Empson, M; Lassere, M; Craig, J; Scott, J (Apr 18, 2005). "Prevention of recurrent miscarriage for women with antiphospholipid antibody or lupus anticoagulant." (PDF). The Cochrane database of systematic reviews (2): CD002859. doi:10.1002/14651858.CD002859.pub2. PMID 15846641.
^ Dolitzky M, Inbal A, Segal Y, Weiss A, Brenner B, Carp H (2006). "A randomized study of thromboprophylaxis in women with unexplained consecutive recurrent miscarriages". Fertil Steril 86 (2): 362–6. doi:10.1016/j.fertnstert.2005.12.068. PMID 16769056.

UpToDate Contents

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1. 抗リン脂質抗体症候群の診断 diagnosis of the antiphospholipid syndrome
2. 抗リン脂質抗体症候群の臨床症状 clinical manifestations of the antiphospholipid syndrome
3. 抗リン脂質抗体症候群の治療 treatment of the antiphospholipid syndrome
4. 静脈血栓塞栓症の患者における理論的根拠と無期限の抗凝固の適応 rationale and indications for indefinite anticoagulation in patients with venous thromboembolism
5. 抗リン脂質抗体症候群の病因 pathogenesis of the antiphospholipid syndrome

English Journal

Severe symptomatic stenosis of visceral and renal arteries leading primary antiphospholipid syndrome diagnosis.

Salaun E1, Bartoli MA1, Soler R1, Khibri H2, Ebbo M2, Bernit E2, Flavian A3, Harlé JR2, Magnan PE1, Sarlon-Bartoli G4.
Annals of vascular surgery.Ann Vasc Surg.2014 Oct;28(7):1796.e9-1796.e13. doi: 10.1016/j.avsg.2014.04.017. Epub 2014 May 21.
Antiphospholipid syndrome (APS) is an autoimmune disorder with combination of at least 1 clinical and 1 laboratory criterion as defined by the SAPPORO statement. Clinical criteria result from vascular thrombosis that can affect artery, venous, or small vessel in any tissue or organ. Arterial stenosi
PMID 24858584

My contribution, my dream - a look at the future of APS.

Koike T.
Lupus.Lupus.2014 Oct;23(12):1332-4. doi: 10.1177/0961203314534306.
We found, in 1994, that the epitope for anticardiolipin antibodies (aCL) developed when β2-glycoprotein I (β2GPI) was adsorbed on polyoxygenated polystyrene plates. We also found, in 2009, that the cleaved form of β2GPI (nicked β2GPI) was bound to angiostatin 4.5 and attenuated its antiangiogeni
PMID 25228741

Antiphospholipid syndrome and the relevance of antibodies to negatively charged phospholipids in diagnostic evaluation.

Tebo A.
Lupus.Lupus.2014 Oct;23(12):1313-6. doi: 10.1177/0961203314544534.
The presence of lupus anticoagulant, moderate-to-high levels of IgG and/or IgM antibodies to beta-2 glycoprotein I or cardiolipin in association with at least one of the two major clinical manifestations (thrombosis and/or pregnancy-related morbidity) are required for a diagnosis of definite antipho
PMID 25228736

Japanese Journal

皮膚血管病変を伴う原発生Sjogren症候群6症例における血中抗リン脂質抗体の検討

木村聡子,竹内そら,相馬良直 [他]
日本皮膚科学会雑誌 = The Japanese journal of dermatology 122(14), 3755-3761, 2012-12
NAID 40019528991

Clinical and magnetic resonance imaging features of multiple sclerosis with autoreactive antibodies in Ishikawa Prefecture, Japan

Takahashi Kazuya,Tanaka Keiko
Journal of Neuroimmunology 250(1-2), 111-114, 2012-09-15
… Systemic lupus erythematosus (SLE)-associated autoantibodies such as anti-Sm antibodies, anti-single stranded DNA antibodies and lupus anticoagulants were only identified in MS, whereas SLE itself is more commonly associated with NMO. …
NAID 120004607553

複数の凝固因子インヒビター偽陽性を示したループスアンチコアグラント・低プロトロンビン血症症候群

森永信吾,井手瑶子,楠本優,緒方美佳,高木一孝,松本恵美子
臨床血液 53(7), 716-720, 2012-07-30
NAID 10030990370

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★リンクテーブル★

リンク元	「ループスアンチコアグラント」「ループス抗凝固因子」「lupus coagulation inhibitor」「LA」
関連記事	「anticoagulant」「lupus」