Antiphospholipid Syndrome or Hughes Syndrome

This article discusses about a pathological condition affecting the immune system of the body resulting in various complications. This condition is called as Antiphospholipid Syndrome. Antiphospholipid Syndrome is also known as Phospholipid Antibody Syndrome and referred to as Hughes Syndrome. Hughes was the physician who had described the causes and symptoms of the Antiphospholipid syndrome.

Antiphospholipid Syndrome or Hughes Syndrome

In this article, we will discuss about some facts about Antiphospholipid Syndrome, what are its causes, symptoms and what are the treatments rendered.

How Is Antiphospholipid Syndrome or Hughes Syndrome Defined?

  • Antiphospholipid Syndrome is a blood disorder caused by Antiphospholipid antibodies.1
  • The hematologic dysfunction is triggered by abnormal immune system. Autoimmune antibodies function as antiphospholipid and causes excessive clotting of blood.
    The example of Antiphospholipid antibody is Anti-cardiolipin antibody or lupus anticoagulant antibodies, which are present in blood.
  • Antiphospholipid Antibodies that can be identified with routine blood examination.

What are Antilipid Antibodies?

  • Antiphospholipid Antibodies are found in people with potentially no underlying disease.
  • Antiphospholipid Antibodies have been detected in very small amounts in the healthy population.
  • Antiphospholipid Antibodies have been detected in blood for some time in association with numerous bacterial or viral conditions like hepatitis, HIV, or malaria infections.
  • Certain medications can also cause production of Antiphospholipid Antibodies in blood. The medications triggers the secretion of Antiphospholipid antibodies are antibiotics, hydralazine and quinine.

Causes of Antiphospholipid Syndrome or Hughes Syndrome

  • The exact cause of Antiphospholipid Syndrome is still unknown.
  • Antiphospholipid Antibodies decreases the levels of a protein called Annexin V, which helps in binding phospholipids, and helps with anticoagulant activity.
  • The reduction in the levels of this protein is believed to be possibly the mechanism behind the increased tendency of blood to clot.

Facts about Antiphospholipid Syndrome or Hughes Syndrome

Some Of The Common Facts About Antiphospholipid Syndrome or Hughes Syndrome Are:

  • This is an autoimmune dysfunction, which has the ability to affect potentially any organ in the body.
  • People with Antiphospholipid Syndrome usually have numerous antibodies to phospholipids in blood.
  • In Antiphospholipid Syndrome there is an increased tendency of the blood to clot.
  • The frontline treatment for Antiphospholipid Antibody is use of anticoagulants.

Causes of Antiphospholipid Syndrome or Hughes Syndrome

Antiphospholipid Syndrome or Hughes Syndrome

Antiphospholipid Syndrome or Hughes Syndrome Causes Blood Clots In Almost All Organs As Follows-

  • Pulmonary Embolism- Development of deep venous thrombosis of pulmonary veins causes pulmonary embolism.
  • DVT of Leg- Deep vein thrombosis of leg veins causes inflammatory phlebitis of leg vein.
  • Miscarriage- Clotting of blood vessels during pregnancy causes miscarriage.
  • Stroke- Blood clots within one of the arterial blood vessels in brain results in stroke.
  • Peripheral Arterial Thrombosis- Causes ischemic changes in leg.
  • Retinal Artery Thrombosis- Causes blindness.4

Symptoms of Antiphospholipid Syndrome or Hughes Syndrome

  • Stroke- Symptoms of stroke are loss of consciousness, headaches, dementia and seizures. The long term effects are as follows-
    • Movement Disorder- Cerebral (brain) cortical dysfunction by ischemia (decrease of blood supply) causes movement disorder.2
    • Cognitive Impairment
    • Sudden Acute Hearing Loss
  • Pulmonary Embolism- Causes chest pain and shortness of breath.
  • DVT- DVT of leg causes swelling and edema of feet, ankle and lower leg. Severe pain while walking and at rest. Severe tenderness and pain following palpation and pressure over inflamed vein of the leg.
  • Miscarriage- Repeated episodes of miscarriages or loss of child before birth in females and also presence of preeclampsia
  • Peripheral Arterial Thrombosis- Severe pain, swelling and tenderness observed around inflamed and thrombosed artery.
  • Coronary Thrombosis3- Severe chest pain like angina, irregular heart beat and low blood pressure observed secondary to coronary artery thrombosis caused by Antiphospholipid Syndrome.
  • Thrombocytopenia- Thrombocytopenia or low platelet count is caused by rapid wear and tear of platelets by Antiphospholipid antibodies.
  • Blindness- Often caused by retinal artery thrombosis.4

Diagnosis of Antiphospholipid Syndrome or Hughes Syndrome

  • Frequent episodes of embolism or thrombosis should further be investigated for by Antiphospholipid antibodies.
  • Miscarriage- If an individual experiences frequent episodes of thrombosis or a female has frequent loss of pregnancy without any known medical cause then the physician may order laboratory tests to look for Antiphospholipid Antibody Syndrome.
  • The physician will order lab tests to specifically look for presence of antibodies to phospholipid-binding proteins. The physician will look for the presence of any of the following three antibodies in the blood.
    • Lupus anticoagulant
    • Anticardiolipin antibody
    • Beta-2 glycoprotein-I
  • Diagnosis- For a confirmatory diagnosis of Antiphospholipid Syndrome, there should be presence of any of the three antibodies mentioned above in the blood at least two times in blood draws which should be conducted at least three months from each other.

Treatment for Antiphospholipid Syndrome or Hughes Syndrome

  • As stated, the frontline treatment for Antiphospholipid Syndrome is anticoagulation so the physicians tend to use anticoagulants to treat Antiphospholipid Syndrome.
  • It is not a cure for the disease but it definitely does help prevent major complications. As an initial treatment course, the physician will start blood thinners in the form of heparin and coumadin.
  • After a few days of combination therapy with heparin and coumadin, the physician will then discontinue heparin but the individual will have to continue coumadin life-long.
  • Aspirin has also been shown to be given for Antiphospholipid Syndrome.
  • Routine blood tests will be drawn when on anticoagulation to make sure the blood has the ability to clot enough to stop bleeding of something like a small cut or bruise.

There are some other treatments for Antiphospholipid Syndrome, which are in the experimental phase. These are:

  • Use of Statins: These are usually used for treating high cholesterol but it has the potential to lessen the risk of increased blood clots.
  • New set of anticoagulants at present in the market is Pradaxa, Xarelto and Eliquis. It is not yet proven that these medications can be used for Antiphospholipid Syndrome.

Home Remedies for Antiphospholipid Syndrome or Hughes Syndrome

If an individual is on an anticoagulant for Antiphospholipid Syndrome then the individual needs to follow the following to prevent bleeding:

  • Avoidance of any form of contact sports that can cause bruise or cut
  • Use of a soft toothbrush
  • Shaving with electric razor
  • Using things like knives, scissors etc. carefully
  • Monitoring the Diet: it is shown that Vitamin K lessens the efficacy of coumadin hence it is necessary to monitor the intake of vitamin K. One should avoid eating large amounts of spinach, lettuce, broccoli, parsley, mustard greens, soybeans etc.
  • Avoid alcohol

References-

1. Revisiting the Molecular Mechanism of Neurological Manifestations in Antiphospholipid Syndrome: Beyond Vascular Damage.

Carecchio M1, Cantello R1, Comi C2.

J Immunol Res. 2014;2014:239398. Epub 2014 Mar 13.

2. Primary antiphospholipid syndrome mimicking demyelinating disorders.

Ioannidis P, Maiovis P, Balamoutsos G, Karacostas D.

J Neuropsychiatry Clin Neurosci. 2014 Apr 1;26(2):E54-5.

3. Recurrent Coronary Thrombosis, Factor V Leiden, Primary Antiphospholipid Sydrome, and HIV.

Santos JL1, Cruz I, Herrero FM, Albarrán C, González Matas JM, Martín Luengo C.

Rev Esp Cardiol (Engl Ed). 2004 Oct;57(10):997-9.

4. Bilateral Central Retinal Vein Occlusion in a patient with Ulcerative Colitis and Antiphospholipid Antibody Syndrome.

Lapsia S1, Meyer R, Abazari A, Usmani K, Gathungu G.

J Pediatr Gastroenterol Nutr. 2014 Apr 23.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: March 24, 2015

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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