Hughes Syndrome: Antiphospholipid Syndrome

Antiphospholipid syndrome (APS), also known as Hughes syndrome, is a disorder of the immune system that causes an increased risk of blood clots.
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• Neurologic Manifestations of Hughes Syndrome
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• What causes antiphospholipid syndrome?
• Antiphospholipid syndrome

Patients who experience thrombosis will normally need to take heparin and warfarin. When the thrombosis clears, they will continue with warfarin. A woman with a diagnosis with APS should plan for pregnancy from before conception. Treatment will start at the beginning of pregnancy and finish after delivery. In an unplanned pregnancy, the effectiveness of treatment may be reduced as it will not begin until several weeks after conception.

Treatment will normally be aspirin, heparin, or both, depending on previous clots of pregnancy complications. Warfarin can cause birth defects, and it is not used during pregnancy. If the patient does not respond to this treatment, intravenous immunoglobulin infusions and corticosteroids, such as prednisone, may be prescribed.

If, by the third trimester, there are no problems, the heparin treatment may stop, but the aspirin treatment may have to continue until the end of the pregnancy. Regular blood tests will need to continue to ensure the blood can still clot enough to stop bleeding if the patient bruises or cuts herself. A doctor will test for antiphospholipid syndrome if the patient has at least one episode of thrombosis or a pregnancy loss.

Sometimes harmless antiphospholipid antibodies may develop for limited periods, because of an infection or some medication, so a second test will be needed to confirm the result. If blood tests reveal abnormal antibodies, the doctor will assess the patient's medical history to determine whether previous symptoms may have been caused by antiphospholipid syndrome.

APS is an autoimmune disorder.

An autoimmune disorder causes antibodies to attack good cells by mistake. If the antibodies attack the phospholipids, this can increase the risk of developing blood clots. The fats and proteins that are attacked are thought to be important in maintaining blood consistency. People with antiphospholipid syndrome produce antibodies that attack either the phospholipids or the blood proteins that bind to the phospholipids.

Why autoimmune disorders happen is unclear, and it is also unclear why some people with abnormal antibodies never develop symptoms.

Genetics factors appear to affect the likelihood of having APS. If a family member has the syndrome, the individual will have a higher risk of developing it themselves. Some people have the antibodies but do not develop signs or symptoms. However, certain triggers can cause the condition to develop in these people.

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Young and middle-aged women are more likely to develop APS, but it can affect either gender and at any age. A patient with APS needs to take all possible measures to lower the risk of developing blood clots. It is important to follow a healthful diet with plenty of fruit and vegetables and low fat and sugar. People who use warfarin need to maintain a stable intake of vitamin K.

Patients should not make any dietary changes or use any supplements of new medications without first consulting their physician. Article last updated by Yvette Brazier on Mon 22 January All references are available in the References tab. Therapeutic Advances in Musculoskeletal Disease 5 6: Catastrophic antiphospholipid antibody syndrome and thrombotic storm. Long term outcome of catastrophic antiphospholipid syndrome survivors. Frequently asked questions about APS answers to questions Valvular heart disease in antiphospholipid syndrome.

Current Rheumatology Reports 15 4: MNT is the registered trade mark of Healthline Media.

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What causes antiphospholipid syndrome?

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Hughes syndrome is thickening of the circulating blood caused by an abnormal immune system. Complications include heart attack, stroke and recurrent miscarriage. Treatment includes medications to thin the blood and prevent platelets from clumping together. Hughes syndrome, or antiphospholipid antibody syndrome APS , is an autoimmune condition that causes thickening of the circulating blood. The immune system produces abnormal blood proteins called antiphospholipid antibodies, which cause blood platelets to clump together.

People with certain autoimmune diseases such as systemic lupus erythematosus SLE are at increased risk of having Hughes syndrome. Without medical treatment, blood clots may lodge in veins or arteries and cause dangerous complications. Any organ or limb can be affected, depending on the site of the blood clot. Hughes syndrome is one of the more common autoimmune conditions, yet it is not as well known as some other diseases of the immune system. It is a lifelong condition and the causes are unclear. There is no cure, but medical treatment can ease symptoms and reduce the risk of complications.

Antiphospholipid antibodies explained The antiphospholipid antibody is an abnormal type of blood protein that is usually found only in people who have certain diseases such as Hughes syndrome or systemic lupus erythematosus. The reason for this overlap is unclear. This autoimmune reaction results in platelets sticking together.

Clumping of platelets causes the build-up of clots. It is not clear what triggers the production of antiphospholipid antibodies. It is thought that about two in every healthy people have mild but detectable levels of antiphospholipid antibodies following illnesses such as hepatitis or malaria.

Some drugs such as cocaine, quinine and antibiotics can also cause the blood to make antiphospholipid antibodies. Symptoms of Hughes syndrome Without treatment, Hughes syndrome can cause many symptoms and complications, including: Migraine headaches Mottled skin tone livedo reticularis Low blood platelet count thrombocytopenia Vein thrombosis Deep vein thrombosis DVT Arterial thrombosis Heart attack Stroke Catastrophic antiphospholipid syndrome.

Complications of pregnancy Hughes syndrome has been identified as a major cause of recurrent miscarriage. The blood of a woman with Hughes syndrome may be too sticky to pass through the tiny blood vessels of the placenta. Miscarriage Fetal death Premature labour. Catastrophic antiphospholipid syndrome This is a rare but extremely life-threatening complication of Hughes syndrome. Typically, a healthy patient with Hughes syndrome suddenly develops multiple clots in various vital organs, including the brain, heart, lungs or liver. The reason for this is unclear.

However, studies show that a percentage of patients have had a recent viral or bacterial infection, which leads some medical researchers to believe that infection may be a trigger. Primary and secondary Hughes syndrome The two types of Hughes syndrome include: Primary — the disease occurs on its own, not in association with an autoimmune condition such as systemic lupus erythematosus.

Most people with Hughes syndrome have the primary type. Secondary — the disease occurs in association with a health problem somewhere else in the body, such as systemic lupus erythematosus. Diagnosis of Hughes syndrome Tests used in the diagnosis of Hughes syndrome include: Medical history Physical examination Blood tests. Treatment for Hughes syndrome There is no cure for Hughes syndrome. Treatment aims to ease symptoms and reduce the risk of complications. Medications to stop platelets from clumping together, such as low-dose aspirin Medications to thin the blood, such as heparin Cortisone drugs to control the inflammation associated with autoimmune diseases such as lupus Drug therapy to control other health problems, such as hypertension high blood pressure or diabetes, that may increase the risk of complications including stroke or heart attack Regular medical check-ups to monitor the medications and reduce the risk of side effects: Ongoing medical treatment allows most people with Hughes syndrome to live long and healthy lives.

However, you may need to take medications for long periods of time, perhaps for the rest of your life.

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Some people are troubled by complications, despite the best of care. Where to get help Your doctor, who can refer you to a specialist such as a haematologist, obstetrician or rheumatologist In an emergency, always call triple zero Musculoskeletal Australia. National Help Line Tel. How long and how high with oral anticoagulant therapy? Send us your feedback. Rate this website Your comments Questions Your details.

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