Lupus Anticoagulant: Causes, Symptoms, Diagnosis & Treatment

Lupus anticoagulant (LA) is an autoimmune antibody that impacts the body’s normal clotting process and increases one’s risk for abnormal blood clots. While its name may make it sound related to the chronic autoimmune condition systemic lupus erythematosus (SLE), LA is actually not connected to SLE specifically. However, LA is more common in people who have SLE or similar disorders.

In this comprehensive guide, we’ll help you understand everything you need to know about this clotting disorder – from causes and risk factors to symptoms, diagnosis, potential complications and available treatment approaches.

What Exactly Is Lupus Anticoagulant?

  • Lupus anticoagulant is an immunoglobulin (antibody) that essentially makes the blood hypercoagulable – meaning it promotes inappropriate clotting.
  • It gets its name from a similar anticoagulant property initially seen in blood samples of people with SLE in the 1940’s. However, unlike what the name suggests, LA is not solely connected to lupus nor is it truly an anticoagulant.
What Is A Lupus Anticoagulant
  • LA antibodies bind to phospholipids in the body, interrupting the normal anticoagulant functions of proteins that depend on phospholipids. This causes an increased tendency of inappropriate clot formation.
  • Estimated prevalence of LA ranges from 1-5% of the general population. However, it’s much more common in those who have other autoimmune disorders like SLE, occurring in over 30% of people with SLE.

So in summary – lupus anticoagulant is a specific type of autoantibody that disrupts normal blood clotting pathways in the body, promoting abnormal clotting. It’s seen more often in some autoimmune disorders but can occur on its own too.

What Causes Lupus Anticoagulant and Who Is At Risk?

Lupus anticoagulant is not associated with any single causative factor. Some potential contributors to developing LA antibodies include:

  • Having another autoimmune disorder, like SLE or antiphospholipid syndrome. Around 30-40% of people with SLE will test positive for LA.
  • Infections, including HIV, hepatitis C virus, syphilis, malaria and others. These infections can prompt the immune system to make LA antibodies.
  • Certain medications, such as antibiotics, can also serve as triggers.
  • Genetic predisposition – some people may inherit a tendency to produce LA antibodies.
  • Pregnancy – LA antibodies are detected in some women only during pregnancy, disappearing after delivery. Reasons are unclear.
  • No identifiable cause in around half of diagnosed cases of LA.

While the causes behind lupus anticoagulant are variable and not definitively established, these factors are believed to increase one’s risk:

  • Having an existing autoimmune disorder, like SLE
  • Age – risk increases as you get older
  • Having genetic variants associated with phospholipid antibodies
  • Vitamin D deficiency
  • Smoking

The takeaway is that LA can occur without any underlying disorder in otherwise healthy people, prompted by one of the potential triggers above. Those at highest risk are people who already have a related autoimmune condition.

Signs and Symptoms of Lupus Anticoagulant

Signs and Symptoms of Lupus Anticoagulant

One of the most perplexing aspects of lupus anticoagulant is that it often causes no signs or symptoms at all, at least initially.

  • Many people have LA antibodies present in their blood but never develop issues from it. It’s often discovered accidentally when getting blood testing done for other reasons.
  • So why treat it then? The main complication LA causes is an increased risk for developing abnormal blood clots since it promotes the body’s clotting mechanisms.
  • Clots can happen in both arteries and veins. Complications arise when clots occur in critical organs and block oxygen-rich blood from reaching those tissues.

Some potential symptoms if clots do start to develop include:

  • Sudden shortness of breath, chest pain – suggesting clots in the lungs
  • Vision changes, headache, weakness on one side of the body – indicating a stroke
  • Severe pain, swelling in one limb – raises concern for a DVT blood clot
  • Seizures or altered mental status
  • Multi-organ failure in severe cases

Clots that develop with LA may happen spontaneously with no clear trigger. Or certain additional risk factors can increase chances of clotting – things like pregnancy, infections, injuries, smoking or taking estrogen therapies.

Bottom line – LA itself causes no direct symptoms. But secondary complications like blood clots can arise, with potentially life-threatening effects if not promptly treated.

How is Lupus Anticoagulant Diagnosed?

Detecting the presence of LA involves blood testing for specific autoantibodies. Diagnosis is made based on the following criteria:

  • Prolonged phospholipid-dependent clotting test times – LA antibodies interfere with these lab test results even though bleeding/clotting times are normal. Repeat testing on a second occasion is needed to confirm.
  • Lack of correction in mixing tests – LA antibodies cause persistently prolonged clotting times on lab assays which fail to correct when the sample is mixed with normal plasma.

Phospholipid-dependent coagulation tests used to detect LA antibodies in blood samples include:

  • aPTT – Activated partial thromboplastin time
  • dRVVT – Dilute Russell viper venom time
  • KCT – Kaolin clotting time
  • Mixing tests using normal plasma

These specialized blood coagulation assays need to remain prolonged and fail to correct on two separate instances about 6-12 weeks apart before making a diagnosis of lupus anticoagulant.

LA testing is complex and it can even be tricky for hematologists to interpret. Results need correlation with each patient’s clinical picture for accurate diagnosis. What’s importantly to note is that LA testing is different from workup for other common clotting abnormalities like factor V Leiden mutation.

What Are Complications of Lupus Anticoagulant?

The hallmark complication lupus anticoagulant causes is inappropriate blood clots forming in the vascular system. This hypercoagulable state stems from the way LA antibodies disable proteins that normally prevent clots from developing unchecked.

Some key facts about risks of thrombosis are:

  • Arterial clots are more common than venous clots
What Are Complications of Lupus Anticoagulant?
  • Up to 1 in 5 people with a CLA antibody will develop clots during their lifetime
  • Certain additional risk factors boost chances of clots

Another major issue is significantly greater risk of pregnancy complications when LA antibodies are present. These include:

  • Miscarriage
  • Intrauterine growth restriction (IUGR)
  • Preeclampsia
  • Placental abruption
  • Stillbirth

The exact reasons are unclear but LA interferes with normal development of the placenta and weakens integrity of blood vessels supplying the fetus. Testing for antiphospholipid antibodies should be part of routine prenatal labs.

Finally, some people with elevated LA levels never experience any symptoms or issues from thrombotic complications. But given risk of life-threatening events is still substantially higher for those affected, treatment is warranted.

Home remedies to treat Lupus Anticoagulant

People suffering from Lupus and other auto-immune disorders which involve joint pain experience relief from drinking a cup of nettle tea or eating stewed nettle leaves daily… Dr. Paul Hamilton

How is Lupus Anticoagulant Treated?

General treatment principles for managing lupus anticoagulant focus on:

  • Preventing first or recurrent blood clots
  • Addressing any related autoimmune disorder
  • Lifestyle measures to minimize other clotting risk factors
  • Preventing pregnancy complications when trying to conceive

The cornerstones of medical treatment are anticoagulant and/or antiplatelet medications to reduce risk of clot recurrence by thinning the blood long term.

Anticlotting Medications Used in Treatment
  • Warfarin – classic anticoagulant usually taken lifelong to prevent clots
  • Heparin then warfarin – for treating existing clots and transitioning to maintenance with warfarin
  • Direct oral anticoagulants (DOACs) like Xarelto, Eliquis, Pradaxa – newer blood thinners sometimes used instead of warfarin
  • Aspirin – inhibits platelet function and has some anticlotting effects
  • Low molecular weight heparin injections – used during high risk situations like pregnancy and surgeries

The decision about whether to anticoagulate with warfarin or DOAC medications, versus using aspirin alone, depends on several factors:

  • History of prior clots
  • Presence of additional clotting risk factors
  • Results of repeat LA antibody blood testing
  • Doctor-patient discussion of medication risks vs benefits

During pregnancy:

  • Prompt treatment with heparin and aspirin reduces chances of complications
  • Warfarin causes birth defects and should be stopped before conceiving

Are there specific treatments that improve survival rates for lupus anticoagulant patients

Based on the search results, there are a few key points regarding treatments that may improve survival rates for patients with lupus anticoagulant:
Anticoagulation Therapy
  • The search results indicate that even with anticoagulant or antiplatelet therapy, many lupus anticoagulant patients still experienced new thrombotic events.
  • In fact, patients receiving anticoagulant and/or antiplatelet therapy had higher incidences of thrombotic events (24%) compared to those not on therapy (13%).
  • This suggests that current standard anticoagulation regimens may not be fully effective at preventing thrombosis in lupus anticoagulant patients.
Preventing Thrombotic Events
  • The search results show that the occurrence of new thrombotic events was the strongest predictor of increased mortality in lupus anticoagulant patients.
  • Patients who developed a new thrombosis had an 8.76-fold higher risk of mortality compared to those without new thrombosis.
  • Therefore, the key to improving survival may be finding more effective ways to prevent thrombotic complications in this patient population.
Other Treatments
  • The search results did not identify any other specific treatments that were shown to improve survival rates for lupus anticoagulant patients.
  • Factors like concomitant autoimmune diseases, baseline anticoagulant treatment, and presence of other antiphospholipid antibodies were not associated with mortality.
In summary, the most important factor appears to be preventing new thrombotic events, as these were the primary driver of increased mortality. However, the current standard anticoagulation therapies do not seem sufficient to fully mitigate this risk. Further research is needed to identify more effective treatment approaches for lupus anticoagulant patients.

Living with Lupus Anticoagulant Long Term

Those diagnosed with lupus anticoagulant need to follow some special considerations when it comes to long-term management, even when on anticoagulant therapies.

Medical Follow Up

  • Have at least annual blood testing to recheck LA antibody levels – these can fluctuate over time
  • See a hematologist knowledgeable about LA for guidance on diagnosis/treatment specifics
  • Carry medical alert information about LA condition
  • Learn to watch for signs of new clots developing & seek urgent care if concerns arise

Lifestyle Measures

  • Stay active – regular gentle exercise promotes healthy circulation
  • Achieve/maintain healthy body weight
  • Don’t smoke or quit if you do – smoking thickens the blood
  • Follow diet tips to avoid vitamin deficiencies that can increase clotting risks

During Times of Additional Risk

Those with lupus anticoagulant need to take extra preventive precautions during situations when clotting risk rises higher, like:

  • Major surgeries
  • Hospitalizations for infections or severe illness
  • Enduring prolonged immobility from long flights or car rides
  • Starting estrogen-containing medications or high-estrogen states like pregnancy

Working closely with your hematology team is key during these high risk periods to adjust anticoagulation dosing accordingly.

What lifestyle changes can help manage lupus anticoagulant

Here are the key lifestyle changes that can help manage lupus anticoagulant:

  • Exercise Regularly: Regular exercise increases blood flow and helps prevent blood clots. Low to moderate intensity exercises like walking, biking, swimming, and yoga are recommended.
  • Quit Smoking: Smoking causes blood vessels to constrict, increasing the risk of blood clots. Quitting smoking is crucial for those with lupus anticoagulant.
  • Moderate Alcohol Intake: Excessive alcohol consumption can also contribute to blood clot formation, so it’s important to drink in moderation.
  • Lose Weight: Excess body fat produces substances that can inhibit the dissolution of blood clots. Maintaining a healthy weight is beneficial.
  • Reduce Vitamin K Intake: Foods high in vitamin K, like leafy greens, broccoli, and prunes, can counteract the effects of blood thinners. Limiting intake of these foods may be helpful.
  • Get Adequate Rest: Fatigue is a common symptom of lupus, so getting enough rest is important to manage the condition.
  • Manage Stress: Stress can trigger lupus flare-ups, so practicing stress management techniques like meditation, deep breathing, and relaxing activities is recommended.

In summary, key lifestyle changes include regular exercise, smoking cessation, moderate alcohol intake, weight management, vitamin K reduction, adequate rest, and stress management. These can help control blood clotting and other symptoms associated with lupus anticoagulant.

What specific exercises are best for managing lupus anticoagulant

Here are the key lifestyle changes and exercises that can help manage lupus anticoagulant:

Recommended Exercises

Low-Impact Activities
  • Walking
  • Swimming
  • Cycling
  • Yoga
  • Tai Chi
  • Pilates
These low-impact exercises are gentle on the joints and muscles, which can be sensitive for those with lupus anticoagulant. They help increase blood flow and prevent blood clots without overstressing the body.
 
Strength Training
  • Squats
  • Alternating lunges
  • Incline push-ups
  • Reverse crunches
  • Bridges
Experts recommend performing these strength exercises 2-3 times per week with 8-12 repetitions. This can help build muscle strength and prevent joint damage.

Other Lifestyle Changes

  • Quit Smoking: Smoking causes blood vessels to constrict, increasing the risk of blood clots. Quitting smoking is crucial.
  • Moderate Alcohol Intake: Excessive alcohol can also contribute to blood clot formation, so drinking in moderation is recommended.
  • Lose Weight: Excess body fat produces substances that can inhibit the dissolution of blood clots, so maintaining a healthy weight is beneficial.
  • Reduce Vitamin K Intake: Foods high in vitamin K, like leafy greens, can counteract the effects of blood thinners, so limiting intake may be helpful.
  • Get Adequate Rest: Fatigue is common with lupus, so getting enough rest is important to manage the condition.
  • Manage Stress: Stress can trigger lupus flare-ups, so practicing stress management techniques is recommended.
In summary, the key is to focus on low-impact, gentle exercises that promote blood flow and muscle strength, while also making lifestyle changes to reduce clotting risk factors. Consulting a healthcare provider to develop a personalized exercise plan is highly advised.

How often should I exercise if I have lupus anticoagulant

If you are using lupus anticoagulant, it is typically recommended that you exercise regularly; nevertheless, it is critical that you listen to your body and alter your exercise program in response to your symptoms. Here are some important things to consider before starting an exercise regime:

  1. Frequency and intensity: Walking, swimming, cycling, yoga, Pilates, and light strength training are all examples of low to moderate-intensity workouts. These exercises can help you gain flexibility, cardiovascular fitness, and muscle strength while reducing joint stress.
  2. Consistency: Maintain consistency in your workout regimen and incorporate a variety of workouts that target all main muscle groups. Continuously challenging your body with new motions and activities can help keep your workouts interesting.
  3. Pacing: Work at your own pace and within your boundaries. If your workouts induce stress or overwhelm you for whatever reason, including a hectic schedule, lessen the intensity or duration of your routines. Allow yourself plenty of time to relax and recover over time.
  4. Symptom Management: Pay special attention to your symptoms. If you notice greater weariness, joint discomfort, or other symptoms while or after exercising, change your routine or take a break. It is critical to listen to your body and modify your training regimen accordingly.

In summary, exercise regularly but at a moderate intensity, be consistent, pace yourself, and listen to your body to manage symptoms effectively. Consult with your healthcare provider to develop a personalized exercise plan.

how many people die of Lupus Anticoagulant annually

Based on the search results, there are no clear statistics on the annual mortality rate specifically due to lupus anticoagulant. However, the results provide some insights into the mortality associated with lupus anticoagulant:

  • In a prospective study of 151 lupus anticoagulant positive patients followed for a median of 8.2 years, 20 patients (13%) died during the observation period
  • The occurrence of new thrombotic events was the strongest predictor of mortality, associated with an 8.76-fold increased risk of death.
  • Compared to an age-, sex-, and study-inclusion-year matched reference population, the lupus anticoagulant positive cohort showed persistently worse survival, with a cumulative relative survival of 87.7% after 10 year.
  • In a separate study of 192 hospitalized COVID-19 patients, the presence of lupus anticoagulant was not associated with higher mortality.

In summary, while lupus anticoagulant appears to be associated with increased mortality compared to the general population, primarily due to thrombotic complications, the exact annual mortality rate is not clearly quantified in the provided search results. More targeted epidemiological studies would be needed to determine the annual mortality specifically attributable to lupus anticoagulant.

Conclusion & Key Takeaways

Lupus anticoagulant is one of several antiphospholipid antibodies that promotes inappropriate blood clotting by interrupting normal anticoagulant pathways. Key facts covered here include:

  • LA is strongly linked to some autoimmune disorders but also occurs independently.
  • No direct symptoms initially but significantly higher risk of eventual vascular blood clots.
  • Confirmed by specialized lab tests for phospholipid-dependent antibodies.
  • Preventive blood thinners are mainstay treatments to reduce chances of first and recurrent clotting events.
  • Even on medication, those with LA need regular medical follow up and clot risk reduction lifestyle measures.

While its name falsely associates it with lupus specifically, lupus anticoagulant is an important risk factor for abnormal clotting identified in some people with various autoimmune or unexplained reasons. Detecting its presence is critical for preventing life-threatening complications like stroke, pulmonary embolism or pregnancy loss.

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