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'''Lupus anticoagulant''' is an [[immunoglobulin]] that interferes with the body's normal clotting mechanism. Despite its name, lupus anticoagulant is not exclusively associated with [[Systemic lupus erythematosus|lupus]], but can be seen in a range of other conditions as well. The presence of lupus anticoagulant is linked to an increased risk of developing blood clots, a condition known as [[Thrombosis|thrombosis]].<ref name="Devreese">{{cite journal |last1=Devreese |first1=K. |last2=Hoylaerts |first2=M. |year=2009 |title=Lupus Anticoagulant (LA) testing: Performance of clinical laboratories assessed by a national survey using lyophilized affinity-purified immunoglobulin with LA activity |journal=Thrombosis and Haemostasis |volume=101 |issue=2 |pages=337–345 |doi=10.1160/TH08-08-0547}}</ref>
==Lupus Anticoagulant==


== Understanding Lupus Anticoagulant ==
Lupus anticoagulant (LA) is an autoimmune disorder characterized by the presence of antibodies that interfere with the normal clotting process. Despite its name, lupus anticoagulant is associated with an increased risk of thrombosis rather than bleeding. It is commonly associated with [[antiphospholipid syndrome]] and can occur in patients with or without [[systemic lupus erythematosus]].


Lupus anticoagulant is one of the antiphospholipid antibodies, which also include anticardiolipin antibodies and anti-beta-2-glycoprotein I antibodies. These antibodies are often associated with [[Antiphospholipid syndrome|antiphospholipid syndrome (APS)]], a disorder that increases the risk of clot formation. APS can occur as a primary disorder or in conjunction with other autoimmune diseases, most commonly lupus.<ref name="Giannakopoulos">{{cite journal |last1=Giannakopoulos |first1=B. |last2=Krillis |first2=S.A. |year=2013 |title=The pathogenesis of the antiphospholipid syndrome |journal=New England Journal of Medicine |volume=368 |issue=11 |pages=1033–1044 |doi=10.1056/NEJMra1112830}}</ref>
===Pathophysiology===
Lupus anticoagulant antibodies target phospholipid-binding proteins, such as [[beta-2 glycoprotein I]] and [[prothrombin]]. These antibodies disrupt the coagulation cascade, leading to a paradoxical increase in clotting tendency. The exact mechanism by which LA causes thrombosis is not fully understood, but it is believed to involve the activation of endothelial cells and platelets, as well as interference with natural anticoagulant pathways.


== Diagnosis of Lupus Anticoagulant ==
===Clinical Manifestations===
Patients with lupus anticoagulant may present with a variety of clinical manifestations, including:


The diagnosis of lupus anticoagulant is made by laboratory testing, often when investigating a patient who has had an unexplained blood clot or recurrent miscarriages. The diagnostic process typically involves a series of [[Coagulation|coagulation]] tests, including the activated partial thromboplastin time (APTT) and the dilute Russell's viper venom time (dRVVT).<ref name="Moore">{{cite journal |last1=Moore |first1=G.W. |year=2014 |title=Recent guidelines and recommendations for laboratory detection of lupus anticoagulants |journal=Thrombosis Journal |volume=12 |issue=1 |doi=10.1186/1477-9560-12-7}}</ref>
* [[Deep vein thrombosis]]
* [[Pulmonary embolism]]
* [[Stroke]]
* [[Recurrent miscarriage]]


== Clinical Significance of Lupus Anticoagulant ==
The presence of LA is a major criterion for the diagnosis of antiphospholipid syndrome, which is characterized by recurrent thrombotic events and pregnancy complications.


Despite its anticoagulant name, the presence of lupus anticoagulant is paradoxically associated with an increased risk of blood clots rather than bleeding. This occurs because lupus anticoagulant interferes with the body's natural anticoagulant pathways. It has been associated with conditions such as deep vein thrombosis, stroke, myocardial infarction, and recurrent miscarriages, particularly in individuals with APS.<ref name="Urbanus">{{cite journal |last1=Urbanus |first1=R.T. |last2=Siegerink |first2=B. |last3=Roest |first3=M.
===Diagnosis===
|last4=Rosendaal |first4=F.R. |last5=de Groot |first5=P.G. |year=2009 |title=Antiphospholipid antibodies and risk of myocardial infarction and ischaemic stroke in young women in the RATIO study: a case-control study |journal=Lancet Neurology |volume=8 |issue=11 |pages=998–1005 |doi=10.1016/S1474-4422(09)70239-X}}</ref>
The diagnosis of lupus anticoagulant involves a series of laboratory tests, including:


== References ==
* Prolonged [[activated partial thromboplastin time]] (aPTT)
* Mixing studies to rule out factor deficiencies
* Confirmatory tests such as the dilute Russell's viper venom time (dRVVT) and the hexagonal phase phospholipid test


Reference 28 <ref name="Devreese">Devreese, K., Hoylaerts, M. (2009). "Lupus Anticoagulant (LA) testing: Performance of clinical laboratories assessed by a national survey using lyophilized affinity-purified immunoglobulin with LA activity". Thrombosis and Haemostasis. 101(2): 337–345. doi:10.1160/TH08-08-0547.</ref>
These tests are designed to detect the presence of LA antibodies and their effect on the coagulation pathway.
Reference 29 <ref name="Giannakopoulos">Giannakopoulos, B., Krillis, S.A. (2013). "The pathogenesis of the antiphospholipid syndrome". New England Journal of Medicine. 368(11): 1033–1044. doi:10.1056/NEJMra1112830.</ref>
Reference 30 <ref name="Moore">Moore, G.W. (2014). "Recent guidelines and recommendations for laboratory detection of lupus anticoagulants". Thrombosis Journal. 12(1). doi:10.1186/1477-9560-12-7.</ref>
Reference 31 <ref name="Urbanus">Urbanus, R.T., Siegerink, B., Roest, M., Rosendaal, F.R., de Groot, P.G. (2009). "Antiphospholipid antibodies and risk of myocardial infarction and ischaemic stroke in young women in the RATIO study: a case-control study". Lancet Neurology. 8(11): 998–1005. doi:10.1016/S1474-4422(09)70239-X.</ref>
Reference 32 <ref name="Miyakis">Miyakis, S., Lockshin
, M.D., Atsumi, T., Branch, D.W., Brey, R.L., Cervera, R., Derksen, R.H., De Groot, P.G., Koike, T., Meroni, P.L. (2006). "International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS)". Journal of Thrombosis and Haemostasis. 4(2): 295–306. doi:10.1111/j.1538-7836.2006.01753.x.</ref>
{{stub}}
== Treatment and Management ==


Treatment primarily involves long-term anticoagulation therapy to reduce the risk of thrombotic events. This typically involves oral anticoagulants such as [[Warfarin|warfarin]], or newer direct oral anticoagulants. In certain cases, such as in pregnancy, low-dose aspirin or heparin may be used.<ref name="Miyakis">{{cite journal |last1=Miyakis |first1=S. |last2=Lockshin |first2=M.D. |last3=Atsumi |first3=T. |last4=Branch |first4=D.W. |last5=Brey |first5=R.L. |last6=Cervera |first6=R. |last7=Derksen |first7=R.H. |last8=De Groot |first8=P.G. |last9=Koike |first9=T. |last10=Meroni |first10=P.L. |year=2006 |title=International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS) |journal=Journal of Thrombosis and Haemostasis |volume=4 |issue=2 |pages=295–306 |doi=10.1111/j.1538-7836.2006.01753.x}}</ref>
===Treatment===
The management of patients with lupus anticoagulant focuses on preventing thrombotic events. Treatment options include:
 
* [[Anticoagulation therapy]] with medications such as [[warfarin]] or [[direct oral anticoagulants]]
* Low-dose [[aspirin]] for patients with a history of pregnancy complications
 
The choice of treatment depends on the patient's clinical history and risk factors for thrombosis.
 
===Prognosis===
The prognosis for patients with lupus anticoagulant varies depending on the presence of other risk factors and the occurrence of thrombotic events. With appropriate management, many patients can lead normal lives, although they may require long-term anticoagulation therapy.
 
==See Also==
* [[Antiphospholipid syndrome]]
* [[Systemic lupus erythematosus]]
* [[Thrombophilia]]
 
{{Medical conditions}}
{{Autoimmune diseases}}
 
[[Category:Hematology]]
[[Category:Autoimmune diseases]]
[[Category:Coagulation system disorders]]

Latest revision as of 12:37, 31 December 2024

Lupus Anticoagulant[edit]

Lupus anticoagulant (LA) is an autoimmune disorder characterized by the presence of antibodies that interfere with the normal clotting process. Despite its name, lupus anticoagulant is associated with an increased risk of thrombosis rather than bleeding. It is commonly associated with antiphospholipid syndrome and can occur in patients with or without systemic lupus erythematosus.

Pathophysiology[edit]

Lupus anticoagulant antibodies target phospholipid-binding proteins, such as beta-2 glycoprotein I and prothrombin. These antibodies disrupt the coagulation cascade, leading to a paradoxical increase in clotting tendency. The exact mechanism by which LA causes thrombosis is not fully understood, but it is believed to involve the activation of endothelial cells and platelets, as well as interference with natural anticoagulant pathways.

Clinical Manifestations[edit]

Patients with lupus anticoagulant may present with a variety of clinical manifestations, including:

The presence of LA is a major criterion for the diagnosis of antiphospholipid syndrome, which is characterized by recurrent thrombotic events and pregnancy complications.

Diagnosis[edit]

The diagnosis of lupus anticoagulant involves a series of laboratory tests, including:

  • Prolonged activated partial thromboplastin time (aPTT)
  • Mixing studies to rule out factor deficiencies
  • Confirmatory tests such as the dilute Russell's viper venom time (dRVVT) and the hexagonal phase phospholipid test

These tests are designed to detect the presence of LA antibodies and their effect on the coagulation pathway.

Treatment[edit]

The management of patients with lupus anticoagulant focuses on preventing thrombotic events. Treatment options include:

The choice of treatment depends on the patient's clinical history and risk factors for thrombosis.

Prognosis[edit]

The prognosis for patients with lupus anticoagulant varies depending on the presence of other risk factors and the occurrence of thrombotic events. With appropriate management, many patients can lead normal lives, although they may require long-term anticoagulation therapy.

See Also[edit]