Anticardiolipin Antibody Syndrome

What is Anticardiolipin Antibody Syndrome?

Persistent elevation of levels of antibodies intended to act against membrane anionic phospholipids such as anticardiolipin [aCL] antibody due to an abnormality in the immune system. Anticardiolipin antibody syndrome is related to antiphospholipid syndrome (APS).

In Anticardiolipin antibody syndrome, clotting disorder is mainly associated, including deep vein thrombosis and even sometimes it is associated with clot formation in the specific organs like the lungs or kidney. The severity of the syndrome can be estimated by the coverage and location of the clot. Clot in the brain can increase the incidence of the cerebral stroke. ^(1,4)

Causes

Anticardiolipin antibody is one of the major types of antiphospholipid antibodies and involve in the association of the reduced levels of prostacyclin. Reduction of prostacyclin leads to a chemical reaction and the capacity of prostacyclin in regards of prevention of the platelet aggregation is diminished and blood becomes clotted inside the vein or arteries. Anticardiolipin antibody is associated with different immune system disorders, including systemic lupus erythematosus.⁽³⁾

Signs & Symptoms

The sign and symptoms of anticardiolipin antibody syndrome are similar as antiphospholipid syndrome, the degree of severity varies with individual to individual and that may include:

• Deep vein thrombosis mainly affect in the major vein present in the leg and the clot can travel from the site of origin to other organs and cause pulmonary embolism (clot ensue in the lungs)
• Different pregnancy related complications like consecutive miscarriages or stillbirths, preterm delivery and preeclampsia (hypertension during pregnancy may be due to fluid accumulation and proteinuria)
• Sudden localized brain tissue necrosis due to insufficient systemic circulation (Stroke)
• Peripheral arterial thrombosis, in which blood clots may form in the arteries of the upper limb or lower limb.

Less associated symptoms include

Problems linked with CNS

Migraine, in which patients complain chronic headache. Other neurological problems like dementia and convulsions is the resultant of restricted blood flow in certain brain regions due to the development of the blood clot.

Skin complaints

Onset of red rashes which have a net like outline, quite similar as lacy outlook (livedo reticularis).

Cardiovascular disease

Damage of the heart valves may associated with anticardiolipin antibody syndrome.

Thrombocytopenia

Decreased level of platelets can cause prolonged bleeding, as there is a lack of sufficient active platelets to form clots. This is quite obvious in the nose and gum bleeding, but rarely petechiae (red patch in the skin due to bleeding insider the epidermal layer) also associated.

Occasional signs and symptoms

• Locomotion related problem like chorea can develop in some individual, in which uncontrollable jerks in the body and limbs occur.
• Cognitive impairment like poor memory power.
• Abrupt audible range becomes low

Probabilities which increase AAS

• Association of autoimmune diseases like Sjogren’s syndrome or systemic lupus erythematosus
• Certain infections like HIV/AIDS, syphilis, Lyme disease and hepatitis C
• Intake of certain medicines, which include hydralazine for hypertension, antiarrythmic drugs like quinidine, anti-convulsion medicine like phenytoin (Dilantin) and antibacterial therapy like amoxicillin.
• Hereditary factors ^(3,4)

Risk Factors

Following are the risk factors which are contributes to worse the symptoms

• Pregnancy
• Prolonged sitting position or restricted movement
• Surgical intervention
• Cigarettes smoking
• Ingestion of oral contraceptive pills
• Hypercholesterolemia ⁽⁴⁾

Diagnosis

A specific test is conducted for cardiolipin antibodies and this helps to identify the reason behind the following conditions:

• Inexplicable thrombotic episodes
• Repetitive miscarriages
• Persistence partial thromboplastin time and for confirmation of the cause dilute Russell viper venom test (DRVVT) test is also combine

The presence of cardiolipin antibodies need to repeat the test after six weeks. This test is conducted for determining the finding is persistent or temporary. The presence of autoimmune disorder usually cause positive test result of cardiolipin antibodies.

Other tests

Sometimes doctors order combinations of test like cardiolipin antibody tests with anti-beta 2 glycoprotein I antibody.

There are 3 types of cardiolipin antibodies such as IgG, IgM and/or IgA, which may present in the blood sample. Frequently test order is provided for detection of IgG and IgM, if the result of these is negative, but the probability of cardiolipin antibody syndrome is present, then test for IgA cardiolipin antibody may be ordered. (5)

Treatment

The major symptom of the anticardiolipin antibody syndrome is thrombus formation, to resolve or disintegrate the clot, the combination of blood thinning (anticoagulant) medications is prescribed.

Heparin & warfarin

Heparin is first line treatment for thrombus disintegration. Heparin is available in vial or ampoule, and given in injection dosage form followed with oral therapy with warfarin. After certain periods, depending upon the symptomatic relieve, doctors may withdraw heparin and only warfferin is continues for the rest of the life.

Aspirin

In some cases, doctors also prescribe low-dose aspirin, as aspirin has blood thinning property.

During the treatment, periodic clotting time estimation is ordered for checking the patient’s blood clotting capacity, as the therapy makes the blood thinner than the normal and it may hamper the normal clotting time and any injury or hemorrhage cause excessive blood loss.

Novel pharmacotherapy

The following drugs are experimentally added to the prescription and give satisfactory result. Some of them are also approved by drug authorities as an anticoagulant therapy.

Statins

• Statins are usually prescribed as anti- hypercholesterolemia, but nowadays it is also incorporated in the prescription for treating the anticardiolipin antibody syndrome, as they provide beneficial effects in reduction of the blood clot formation and prevent cardiovascular disease.

Novel anticoagulants

Some newly invented oral drugs get approval as anticoagulants, which include dabigatra, rivaroxaban and apixaban, though these drugs are not specific treatments for the cardiolipin antibody syndrome and not recommended for pregnant or lactating mothers.

Rituximab

This drug is used for immune system disorder, but does not have sufficient data for treating the cardiolipin antibody syndrome. ⁽⁴⁾

References

1. Teresa G Berg (2015); Antiphospholipid Syndrome and Pregnancy;: http://emedicine.medscape.com/article/261691-overview
2. What Are the Signs and Symptoms of Antiphospholipid Antibody Syndrome?; National heart, lung and Blood Institute;: http://www.nhlbi.nih.gov/health/health-topics/topics/aps/signs
3. William C. Shiel Jr., (2015) Antiphospholipid Syndrome;: http://www.medicinenet.com/antiphospholipid_syndrome/page2.htm
4. Mayo clinic (2015); Antiphospholipid syndrome;: http://www.mayoclinic.org/diseases-conditions/antiphospholipid-syndrome/basics/treatment/con-20028805
5. Cardiolipin Antibodies (2012); Lab Tests Online;: https://labtestsonline.org/understanding/analytes/cardiolipin/tab/test/