Lupus anticoagulant: Difference between revisions

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{{Short description|A type of antibody associated with increased risk of thrombosis}}
{{Infobox medical condition (new)  
{{Infobox medical condition (new)  
| name            = Lupus anticoagulant  
| name            = Lupus anticoagulant  
| image          = 
| field          = [[Hematology]], [[Immunology]]
| caption        = 
| pronounce      = 
| field          =
| synonyms        = Lupus antibody, LA, LAC, lupus inhibitors
| synonyms        = Lupus antibody, LA, LAC, lupus inhibitors
| symptoms        =  
| symptoms        = [[Thrombosis]], [[recurrent miscarriage]], prolonged [[activated partial thromboplastin time]] (aPTT)
| complications  =  
| complications  = [[Deep vein thrombosis]], [[pulmonary embolism]], [[stroke]], [[pregnancy loss]]
| onset          =  
| onset          = Variable (often detected during clotting evaluation)
| duration        =  
| duration        = Can be transient or persistent
| types          =  
| types          = Associated with [[Antiphospholipid syndrome]]
| causes          =  
| causes          = Autoantibodies targeting phospholipid-binding proteins (e.g., β2-glycoprotein I)
| risks          =  
| risks          = [[Systemic lupus erythematosus]] (SLE), [[HIV]], [[hepatitis C]], certain medications
| diagnosis      =  
| diagnosis      = Positive testing on at least two occasions, 12 weeks apart; includes dRVVT, aPTT, and LA-sensitive assays
| differential    =  
| differential    = Other causes of thrombosis, [[protein C deficiency]], [[antithrombin III deficiency]], [[factor V Leiden]]
| prevention      =  
| prevention      = Risk management for thrombosis in high-risk individuals
| treatment      =  
| treatment      = Anticoagulation therapy
| medication      =  
| medication      = [[Warfarin]], [[heparin]], [[low-molecular-weight heparin]]
| prognosis      =  
| prognosis      = Variable; higher risk of complications with antiphospholipid syndrome
| frequency      =  
| frequency      = Present in ~1–5% of general population; higher in autoimmune diseases
| deaths          =  
| deaths          = Related to thrombotic complications if untreated
}}
}}
'''Lupus anticoagulant''' is an [[immunoglobulin]]<ref name="JoussenGardner2007">{{cite book|author1=Antonia Joussen|author2=T.W. Gardner|author3=B. Kirchhof|title=Retinal Vascular Disease|url=https://books.google.com/books?id=XBiCIhGenxsC&pg=PA430|accessdate=29 June 2010|date=23 October 2007|publisher=Springer|isbn=978-3-540-29541-9|pages=430–}}</ref> that binds to [[phospholipid]]s and [[protein]]s associated with the cell membrane. Its name is a [[misnomer]], as it is actually a [[thrombosis|prothrombotic]] antibody. Lupus anticoagulant in living systems cause an increase in inappropriate blood clotting. The name derives from their properties in vitro, as these antibodies increase laboratory coagulation tests such as the [[Partial thromboplastin time|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.
'''Lupus anticoagulant''' is an [[autoantibody]] associated with an increased risk of [[thrombosis]], or blood clot formation. Despite its name, lupus anticoagulant is not related to [[lupus erythematosus]] and does not cause bleeding. Instead, it interferes with the normal clotting process, leading to a paradoxical increase in clotting risk.


==Pathophysiology==
Lupus anticoagulant is a type of [[antiphospholipid antibody]] that targets [[phospholipids]] and [[phospholipid-binding proteins]] in the cell membrane. These antibodies interfere with the coagulation cascade, particularly affecting the [[prothrombin]] and [[activated partial thromboplastin time]] (aPTT) tests, which are used to assess blood clotting. The presence of lupus anticoagulant prolongs these clotting times in vitro, but in vivo, it predisposes individuals to thrombosis.
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==Clinical Significance==
Lupus anticoagulant is a key component of the [[antiphospholipid syndrome]] (APS), a disorder characterized by recurrent [[venous]] or [[arterial thrombosis]] and [[pregnancy complications]] such as [[recurrent miscarriage]]. Patients with lupus anticoagulant may experience [[deep vein thrombosis]], [[pulmonary embolism]], [[stroke]], or other thrombotic events.


The condition was first described by hematologist [[C. Lockard Conley]].<ref>{{cite journal|last=Conley|first=C. Lockard|year=1952|title=A hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erythematosus|journal=Journal of Clinical Investigation|volume=31|pages=621–622|doi=10.1172/JCI102648|pmc=436459|pmid=14938435}}</ref><ref name="Lock">{{cite news|url=http://www.hopkinsmedicine.org/hmn/S06/conley.cfm|title=Lock Conley looks back and blushes|date=Spring–Summer 2006|work=Hopkins Medicine|accessdate=5 December 2013|archive-url=https://web.archive.org/web/20130621180626/http://www.hopkinsmedicine.org/hmn/S06/conley.cfm|archive-date=21 June 2013|url-status=dead}}</ref>
==Diagnosis==
The diagnosis of lupus anticoagulant involves a series of blood tests. Initial screening includes the [[activated partial thromboplastin time]] (aPTT) and the [[dilute Russell's viper venom time]] (dRVVT). If these tests are prolonged, further confirmatory tests are performed, such as mixing studies and phospholipid neutralization procedures, to confirm the presence of lupus anticoagulant.


==Terminology==
==Management==
Both words in the term "lupus anticoagulant" can be misleading:
Management of patients with lupus anticoagulant focuses on reducing the risk of thrombosis. This often involves the use of [[anticoagulant]] medications such as [[warfarin]] or [[heparin]]. In patients with antiphospholipid syndrome, long-term anticoagulation may be necessary. During pregnancy, low-dose [[aspirin]] and [[heparin]] are commonly used to prevent complications.


* 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 [[kidney failure]], amongst other complications). People with lupus erythematosus are more likely to develop a lupus anticoagulant than the general population.
==Related pages==
* The term "anticoagulant" accurately describes its function [[in vitro]]. However [[in vivo]], it functions as a ''[[procoagulant]]''.<ref name="urlUntitled Document">{{cite web |url=http://dermatology.wustl.edu/dermsub/caseofmonth/11-2005a.html |title=wustl.edu |work= |accessdate=2009-02-17 |archive-url=https://web.archive.org/web/20080821152206/http://dermatology.wustl.edu/dermsub/caseofmonth/11-2005a.html |archive-date=2008-08-21 |url-status=dead }}</ref>
* [[Antiphospholipid syndrome]]
 
* [[Thrombosis]]
== Workup ==
* [[Autoantibody]]
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]].
* [[Coagulation]]
 
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 reassessed. 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 presence of an inhibitor such as the lupus anticoagulant is excluded, and instead a deficient quantity of clotting factor (that is replenished by the normal plasma) is likely.
 
If the mixing test indicates an inhibitor, diagnosis of a lupus anticoagulant is then confirmed with prolonged [[phospholipid]]-sensitive functional clotting testing, such as the [[dilute Russell's viper venom time]], or the [[Kaolin clotting time]]. As a further confirmation, a second test with the addition of excess phospholipid will correct the prolongation (conceptually known as "phospholipid neutralization"), 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]].<ref name="pmid1782567">{{cite journal |vauthors=Viard JP, Amoura Z, Bach JF | title = [Anti-beta 2 glycoprotein I antibodies in systemic lupus erythematosus: a marker of thrombosis associated with a circulating anticoagulant] | language = French | journal = Comptes Rendus de l'Académie des Sciences, Série III | volume = 313 | issue = 13 | pages = 607–12 | year = 1991 | pmid = 1782567 | doi = }}</ref>
 
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.<ref name="pmid15846641">{{cite journal|last=Empson|first=M|author2=Lassere, M |author3=Craig, J |author4= Scott, J |title=Prevention of recurrent miscarriage for women with antiphospholipid antibody or lupus anticoagulant.|journal=The Cochrane Database of Systematic Reviews|date=Apr 18, 2005|issue=2|pages=CD002859|pmid=15846641|doi=10.1002/14651858.CD002859.pub2|url=http://apps.who.int/rhl/reviews/CD002859.pdf}}</ref>
 
Thrombosis is treated with [[anticoagulant]]s ([[Low molecular weight heparin|LMWHs]] and [[warfarin]]).<ref>{{cite journal |vauthors=Dolitzky M, Inbal A, Segal Y, Weiss A, Brenner B, Carp H |title=A randomized study of thromboprophylaxis in women with unexplained consecutive recurrent miscarriages |journal=Fertil Steril |volume=86 |issue=2 |pages=362–6 |year=2006 |pmid=16769056 |doi=10.1016/j.fertnstert.2005.12.068}}</ref>
 
==References==
{{Reflist}}


== External links ==
== External links ==
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{{Autoantibodies}}
{{Autoantibodies}}
 
{{stub}}
[[Category:Coagulation system]]
[[Category:Hematology]]
[[Category:Autoimmune diseases]]
[[Category:Coagulation system]]
[[Category:Coagulation system]]
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Latest revision as of 15:45, 24 March 2025

A type of antibody associated with increased risk of thrombosis


Lupus anticoagulant
[[File:|250px|alt=|]]
Synonyms Lupus antibody, LA, LAC, lupus inhibitors
Pronounce N/A
Field Hematology, Immunology
Symptoms Thrombosis, recurrent miscarriage, prolonged activated partial thromboplastin time (aPTT)
Complications Deep vein thrombosis, pulmonary embolism, stroke, pregnancy loss
Onset Variable (often detected during clotting evaluation)
Duration Can be transient or persistent
Types Associated with Antiphospholipid syndrome
Causes Autoantibodies targeting phospholipid-binding proteins (e.g., β2-glycoprotein I)
Risks Systemic lupus erythematosus (SLE), HIV, hepatitis C, certain medications
Diagnosis Positive testing on at least two occasions, 12 weeks apart; includes dRVVT, aPTT, and LA-sensitive assays
Differential diagnosis Other causes of thrombosis, protein C deficiency, antithrombin III deficiency, factor V Leiden
Prevention Risk management for thrombosis in high-risk individuals
Treatment Anticoagulation therapy
Medication Warfarin, heparin, low-molecular-weight heparin
Prognosis Variable; higher risk of complications with antiphospholipid syndrome
Frequency Present in ~1–5% of general population; higher in autoimmune diseases
Deaths Related to thrombotic complications if untreated


Lupus anticoagulant is an autoantibody associated with an increased risk of thrombosis, or blood clot formation. Despite its name, lupus anticoagulant is not related to lupus erythematosus and does not cause bleeding. Instead, it interferes with the normal clotting process, leading to a paradoxical increase in clotting risk.

Pathophysiology[edit]

Lupus anticoagulant is a type of antiphospholipid antibody that targets phospholipids and phospholipid-binding proteins in the cell membrane. These antibodies interfere with the coagulation cascade, particularly affecting the prothrombin and activated partial thromboplastin time (aPTT) tests, which are used to assess blood clotting. The presence of lupus anticoagulant prolongs these clotting times in vitro, but in vivo, it predisposes individuals to thrombosis.

Clinical Significance[edit]

Lupus anticoagulant is a key component of the antiphospholipid syndrome (APS), a disorder characterized by recurrent venous or arterial thrombosis and pregnancy complications such as recurrent miscarriage. Patients with lupus anticoagulant may experience deep vein thrombosis, pulmonary embolism, stroke, or other thrombotic events.

Diagnosis[edit]

The diagnosis of lupus anticoagulant involves a series of blood tests. Initial screening includes the activated partial thromboplastin time (aPTT) and the dilute Russell's viper venom time (dRVVT). If these tests are prolonged, further confirmatory tests are performed, such as mixing studies and phospholipid neutralization procedures, to confirm the presence of lupus anticoagulant.

Management[edit]

Management of patients with lupus anticoagulant focuses on reducing the risk of thrombosis. This often involves the use of anticoagulant medications such as warfarin or heparin. In patients with antiphospholipid syndrome, long-term anticoagulation may be necessary. During pregnancy, low-dose aspirin and heparin are commonly used to prevent complications.

Related pages[edit]

External links[edit]


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