Written by:
Courtney Battaglia BSN, RN
August 14, 2024
Understanding Antiphospholipid Syndrome
Antiphospholipid syndrome (APS) is a rare autoimmune disease that causes the body to abnormally form blood clots. There are about 40 to 50 cases per 100,000 people, and women are three to four times more likely to have APS than men. Patients with systemic autoimmune conditions, like lupus, may also get APS.
When someone has APS, the immune system makes antibodies that attack proteins in the blood. These proteins bind to phospholipids, which are important for cell membranes to work correctly and provide protection. When the body starts fighting normal proteins, blood clotting starts. Blood clots then form in the vessels. APS can also cause problems for pregnant women, like frequent miscarriages.
APS can be further grouped as primary or secondary. Primary APS occurs on its own, without another autoimmune disorder. Secondary APS occurs with another autoimmune disease, like lupus.
Risk Factors for Antiphospholipid Syndrome
Researchers don’t exactly know why people develop APS, but there are some known risk factors for the condition, including:
Having another autoimmune disease, like lupus
Family history of the disease
Female sex
Bacterial or viral infections, like HIV or Lyme disease
Use of some drugs, including antibiotics and anti-seizure medicines
Diagnosing Antiphospholipid Syndrome
To diagnose APS, clinicians ask about your health, do a physical check-up, and run lab tests. The criteria for diagnosing APS include at least one symptom, like a blood clot or complications with pregnancy, and a lab test to confirm that antibodies associated with the condition are in your blood.
The lab findings will have one of these antibodies present:
Lupus anticoagulant (LAC)
Anticardiolipin antibodies (aCL)
Beta2-glycoprotein I antibodies
If a lab test shows positive results, you’ll take another test 12 weeks later. The test is repeated because medications or infections can cause antibodies to appear, leading to a false-positive result.
In 2023, the American College of Rheumatology and the European Alliance of Associations for
Rheumatology released new criteria for diagnosing APS. These changes include a list of more detailed symptoms and improved lab tests, which help clinicians better diagnose APS.
Antiphospholipid Syndrome Symptoms
APS can cause a variety of symptoms due to blood clots forming in different parts of the body. The severity and type of symptoms can vary widely. Common symptoms include blood clots, problems during pregnancy, and issues with the brain and nerves. The symptoms can be different for each person and can affect many parts of the body.
Blood Clots
Patients with APS tend to get blood clots often, even while taking blood thinners. Two types of blood clots that often occur in patients with APS include DVT (deep vein thrombosis, a blood clot in a deep vein) and PE (pulmonary embolism – a blood clot in the lungs). DVT can cause pain and swelling in the affected part of the body, while PEs often cause shortness of breath, chest pain, and fatigue.
Pregnancy Complications
APS can cause frequent miscarriages in the second or third trimester of pregnancy. Other pregnancy-related problems might include slower fetal growth, fetal distress, problems with the placenta, and preclampsia.
Neurological
A blood clot in the brain can lead to a stroke or transient ischemic attack (TIA). A TIA can cause symptoms similar to a stroke that lasts for a short time. Sometimes, patients may also have seizures or muscle movements that they can’t control. A clot blocking the retinal artery or vein in your eye can affect vision.
APS symptoms can be different for each person. Some patients might not have symptoms and only have antibodies in their blood. If multiple organs are affected, some patients may have more severe symptoms.
Treating Antiphospholipid Syndrome
The goal of APS treatment is to prevent more blood clots from forming and stop current blood clots from getting larger. Treating APS involves using blood thinners, also called anticoagulants. The type of blood thinner used, and the length of treatment, will depend on each patient’s situation.
Treatment options may include:
Warfarin to prevent clots
Aspirin or clopidogrel (Plavix®) to make the blood less sticky and prevent clots
Pregnant women might need heparin, low-dose aspirin, corticosteroids, or a type of medicine called intravenous (IV) immunoglobulin to help control the immune system and prevent miscarriages
Patients with antibodies in their blood but no symptoms may need aspirin
Direct oral anticoagulants (DOACs), which include blood thinners like apixaban (Eliquis®) and rivaroxaban (Xarelto®), are less effective than warfarin in preventing recurrent blood clots in patients with APS – that’s why warfarin is typically used instead.
Some patients who are at risk of APS but don’t have a diagnosis may need to change their diet, drink more water, and increase exercise. Quitting smoking is also strongly recommended since smoking harms blood vessels (and many other parts of the body).
The bottom line: APS is a rare autoimmune disease that makes blood more likely to clot. While there’s no cure, treatment—usually with blood thinners—can help prevent further complications.
Leg Pain After a DVT: Exploring Causes and Finding Relief
Deep vein thrombosis (DVT) is a blood clot that forms in a deep vein, blocking blood flow. Some patients experience pain after a DVT. Typically, the pain goes away once the clot has dissolved. But sometimes, leg pain can stick around for a while after a DVT, which can be frustrating. This ongoing pain is often a symptom of a condition called post-thrombotic syndrome, or PTS.
PTS causes chronic pain in some patients after a DVT. Not everyone will experience PTS – it impacts approximately 20-50% of people who’ve had a DVT.
Why Pain Occurs After a DVT
A DVT in the legs can damage the valves in veins or the veins themselves. Veins bring blood back to the heart from the tissues. The veins in your extremities, like the legs, use muscle movements to squeeze and help push the blood back to the heart. In your veins, there are valves that act like doors to prevent blood from flowing backward. When a DVT forms in the veins, it acts as a plug and blocks the normal flow of blood. This blockage allows fluid to collect in the veins.
As the fluid builds up, it raises the pressure inside the veins. The veins then begin to widen and become weak. Because of the widening, the typically closed valves can no longer shut properly. Blood flow back to the heart decreases. The fluid builds up, leading to swollen tissues, inflammation, and pain.
Some blood clots heal by turning into scar tissue. This scar tissue can also damage the valves in the vein. Scar tissue affects the valves’ ability to function correctly, causing more fluid to collect in the legs. Sometimes, the vein’s wall can become too narrow from the scar tissue. When the vein narrows, the flow of blood back to the heart is reduced, leading to increased fluid buildup, pressure, and pain.
Damage to the leg veins after a DVT can lead to chronic venous insufficiency, a condition where the veins in the legs aren’t able to effectively return blood to the heart.
Post-Thrombotic Syndrome Risk Factors
Not everyone who has had a DVT will develop PTS. Factors that increase the chance of getting PTS include:
Obesity
Female sex
Use of estrogen therapy
Recurrent blood clots in the same leg
A blood clot that forms in a vein in the upper leg or pelvis
Finding Relief from Leg Pain
There isn’t a cure for PTS, but there are things you can do to manage it and reduce pain.
Medical Treatments
PTS is first treated with compression therapy. Compression therapy makes the veins narrower, which helps to improve the valves’ function. When the valves close properly, blood flow returns to the heart, and leg swelling decreases. Compression therapy may include:
Bandaging: Short-stretch bandages help the calf muscles work better when you walk, improving blood flow back to the heart. Long-stretch bandages, like ACE wraps, can lose their tightness when you walk and need to be changed often.
Elastic compression stockings (ECS): Some patients use ECS to reduce swelling. These stockings come in different levels of compression or tightness. Your healthcare team can help you choose the right one. They come in knee-high or thigh-high lengths and help improve blood flow by applying steady pressure.
Adjustable compression wrap devices: These devices use fasteners like Velcro and allow the patient to adjust the wrap when it feels loose.
Intermittent pneumatic compression (IPC): This type of therapy uses a pump to fill and empty air from hollow plastic leggings worn by the patient. IPC helps by gently squeezing the legs to push blood and fluid out of the tissues. It’s for patients who can’t use compression stockings or when the stockings don’t work.
Surgical procedures may be an option when other treatments don’t work and can improve symptoms. A damaged valve can be repaired through reconstruction, known as valvuloplasty. When a valve has severe damage, it can be repaired by transplanting a healthy valve from a different vein in the arm. If a vein is too narrow, stents can be inserted to widen the vein, increasing blood flow.
Home Remedies and Lifestyle Changes
If you have symptoms of PTS, there are some ways to manage them effectively at home. If the veins and valves are weak, blood doesn’t move back to the heart efficiently, causing more pressure, swelling, and pain. Since your legs are furthest from your heart, fluid can easily collect because of gravity. Here are some tips to reduce pressure in your legs:
Stay active: Walking and other activities can help the calf muscles move blood back to the heart.
Use a footrest when you’re sitting: Even a little elevation can help relieve symptoms and decrease pressure.
Elevate your legs when lying down: Place a firm pillow under your legs to keep them above the level of your heart – but avoid putting pillows under your knees since that can block one of the main leg arteries.
Maintain a healthy body weight: Extra weight can increase stress on the circulatory system, raising venous pressure.
Don’t be in the heat for too long: Heat can cause veins to increase in size, causing more fluid to collect inyour legs, which can worsen symptoms of leg heaviness and swelling.
Decrease the amount of salt in your diet: Large amounts of sodium in your tissues can cause more swelling.
Normal Pain vs. Concerning Pain
Everyone feels pain differently. Patients with PTS will have some degree of moderate-to-severe pain. PTS pain symptoms can include:
Aches or cramping
Feelings of leg heaviness
Paresthesia, or feeling of pins and needles
A clinician should evaluate leg pain that occurs suddenly, is severe, or won’t go away. Contact your clinician if you experience other symptoms with leg pain, such as a fever, or a change in skin color or temperature (like coolness).
Ongoing pain after a DVT can be frustrating and burdensome – but there are ways to help manage the symptoms. Keep in contact with your medical team and have open conversations about how your symptoms are impacting your life.
Prefer to read or print?
If you’d like to read this month’s edition of The Beat offline, or keep a copy for later, you can download the full PDF version ->
