Antiphospholipid syndrome (APS) is an uncommon systematic autoimmune disease where the body’s immune system mistakenly creates antibodies which attacks its own tissues. It is also known as an acquired pre-thrombotic autoimmune condition. These antibodies can cause blood clots (thrombosis) in both the arteries and veins. Another cause of APS can be an underlying autoimmune disorder however it can also develop without any causal factors.

APS is classed as a rare condition however it is one of the more common acquired causes of hypercoagulability. In 2019 a study found the estimated prevalence of APS to be 40-50 persons per 100,000, with the most recurrent clinical indicator being deep vein thrombosis (DVT). The annual incidence is 1 to 2 per 100,000 people, with one study of 1000 participants finding APS is typically diagnosed in younger individuals with only 12.7% being over the age of 50.

Thrombosis can form in the legs, lungs, kidneys and spleen, and can lead to a heart attack or stroke. Cardiovascular disease is the leading cause of morbidity and mortality in people with APS, which is driven by thrombo-inflammation and atherothrombosis mechanisms.

Risk factors include:

• More common in women than men
• Having another autoimmune disorder (for example Lupus)
• Pregnancy
• Immobility (bed rest or sitting during a flight)
• Surgery
• Cigarette smoking
• Oral contraception
• Hormone replacement therapy (HRT)
• High cholesterol and triglycerides levels

Signs and symptoms

Some people may not have any signs or symptoms, however common indications can include:

• Blood clots – deep vein thrombosis (DVT) causing pain, swelling and redness.
• Pregnancy complications – repeated miscarriages or stillbirths, high blood pressure (preeclampsia), and premature delivery.
• Stroke – this can occur in a young person and/or someone with no known risk factors for stroke, such as cardiovascular disease.
• Transient ischemic attack
• Rash – red and lacy, net-like pattern.

Less common signs and symptoms may include:

• Neurological – chronic headaches, migraines and dementia. Seizures are possible when a blood clots blocks blood flow to parts of the brain.
• Cardiovascular disease – APS can damage heart valves.
• Low blood platelet count (thrombocytopenia) – can cause bleeding from the nose, gums, and into the skin, which will look like small red spots.

One research case report from 2021 found that recurrent abdominal pain as the first clinical manifestation of APS. The patient had a history of two miscarriages and after a CT and lab test, APS was diagnosed.

There is no cure for this rare condition, therefore medications to relieve any symptoms and to help reduce the risk of blood clots is the best treatment option. If you are taking anticoagulants or blood thinning medications, some self-care recommendations include:

• Avoid contact sports or activities which may cause bruising or injury.
• Use a soft toothbrush.
• Shave with an electric razor.
• Women should avoid using oestrogen therapy for contraception or menopause.

For the management of thrombotic APS, research suggests reducing the risk of blood clots with anticoagulant therapy. As APS is also associated with cardiovascular disease, reducing risk factors such as hypertension, dyslipidemia and obesity would be beneficial.

Please talk to your GP for the best treatment options and therapy for you.

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References

• Antiphospholipid syndrome - Symptoms and causes - Mayo Clinic
• Bolla, E., Tentolouris, N., Sfikakis, P.P. & Tektonidou, M.G. (2023). Metabolic syndrome in antiphospholipid syndrome versus rheumatoid arthritis and diabetes mellitus: Association with arterial thrombosis, cardiovascular risk biomarkers, physical activity and coronary atherosclerotic plaques. Front. Immunol. 13:1077166. doi:10:3389/fimmu.2022.1077166
• Knight, J.S., Branch, D.W., & Ortel, T.L. (2023). Antiphospholipid syndrome: advances in diagnosis, pathogenesis, and management. BMJ, 380: e069717. doi:10.1136/bmj-2021-069717
• Petri, M. (2020). Antiphospholipid syndrome. Translational Research, 225. 70-81.
• Zou, X., Fan, Z., Zhao, L. et al. (2021). Gastrointestinal symptoms as the first manifestation of antiphospholipid syndrome. BMC Gastroenterol 21, 148.