Antiphospholipid Syndrome


In the case of antiphospholipid syndrome, the immune system unintentionally produces antibodies that assault bodily tissues. Blood clots may develop in arteries and veins as a result of these antibodies.

Legs, lungs, and other organs including the kidneys and spleen can develop blood clots. Heart attacks, strokes, and other illnesses can result from clots. Antiphospholipid syndrome during pregnancy can potentially lead to stillbirth and miscarriage. Some patients with the illness show no symptoms at all.

Although there is no known treatment for this unusual illness, several drugs can lower the risk of miscarriage and blood clots.

Antiphospholipid Syndrome: Causes

Antiphospholipid syndrome results when the immune system unintentionally generates antibodies that increase blood clotting risk. Normally, antibodies defend the body from outside invaders like germs and viruses.

  • An underlying ailment such as an autoimmune disorder can contribute to antiphospholipid syndrome. The condition might also appear without any underlying reason.

  • It is unclear why patients produce anti-phospholipid autoantibodies. These autoantibodies are presumably produced in response to an environmental stimulus, such as an infection that occurs in a person who has a genetic predisposition to the disease.

  • APL may stay in the bloodstream for a very long period, although thrombotic events only infrequently happen as a result. APL increases the chance of blood clotting, however, thrombosis often happens when additional factors like extended inactivity (such as being confined to bed), surgery, or pregnancy are present.

  • Hypertension, obesity, smoking, atherosclerosis (hardening of the arteries), estrogen usage (birth control), and a systemic autoimmune illness are additional risk factors for thrombosis.

Antiphospholipid Syndrome: Symptoms

The major symptoms include −

  • Leg clots in the blood (DVT). DVT symptoms include discomfort, redness, and edema. The lungs may get these clots (pulmonary embolism).

  • Miscarriages or stillbirths that keep happening. Premature birth and extremely high blood pressure (preeclampsia) are other pregnancy risks.

  • Stroke. A young individual with antiphospholipid syndrome who has no recognized risk factors for cardiovascular illnesses may get a stroke.

  • Momentary ischemia attack (TIA). Similar to a stroke, a TIA typically only lasts a few minutes and has no lasting effects.

  • Rash. A red rash with a lacy, net-like appearance might appear on certain persons.

Less typical symptoms and indications include −

  • Signs of the nervous system. When a blood clot prevents blood flow to certain areas of the brain, convulsions, dementia, and chronic headaches, including migraines, may result.

  • The cardiovascular system. Heart valve injury from antiphospholipid syndrome is possible.

  • Low levels of blood platelets (thrombocytopenia). Periods of bleeding, especially from the gums and nose, may result from this reduction in blood cells essential for clotting. Patches of tiny red dots will show up where bleeding has penetrated the skin.

When to Visit a Doctor?

If you have inexplicable bleeding from your nose or gums, abnormally heavy menstruation, bright red or coffee-ground-colored vomit, black, tarry, or bright red stools, or unexplained stomach pain, get in touch with your doctor straight once.

If you exhibit symptoms of or need immediate medical attention for −

  • Stroke. Your face, arm, or leg may become suddenly numb, weak, or paralyzed as a result of a brain clot. You might experience severe headaches, vision problems, and difficulties hearing or interpreting speech.

  • Respiratory embolism. You can notice abrupt breathlessness, chest discomfort, and blood-stained mucus when a clot lodges in your lung.

  • Thoracic deep vein (DVT). DVTs might show signs including edema, redness, or discomfort in the arm or leg.

Antiphospholipid Syndrome: Risk Factors

Women experience antiphospholipid syndrome more frequently than males. Antiphospholipid syndrome is more likely in those who also have an autoimmune disorder like lupus.

Antiphospholipid syndrome-related antibodies might exist without causing any symptoms or indicators to appear. But if you have these antibodies, you're more likely to get blood clots, especially if you −

  • Becoming pregnant

  • Are temporarily immobilized, such as while receiving bed rest or seated for a lengthy flight

  • Undergo surgery

  • Puff on a cigarette

  • Utilize estrogen treatment or oral contraceptives to treat menopause.

  • Have high levels of triglycerides and cholesterol

Antiphospholipid Syndrome: Diagnosis

Your doctor can arrange blood tests to look for clotting and the presence of the antibodies linked to antiphospholipid syndrome if you've experienced bouts of blood clots or miscarriages that aren't explained by established medical illnesses.

Antibodies must show up in your blood at least twice, in tests done 12 weeks or more apart, to support a diagnosis of antiphospholipid syndrome.

Antiphospholipid antibodies may be present without any symptoms or indicators ever appearing. Antiphospholipid syndrome is only identified when these antibodies result in medical issues.

The presence of lupus anticoagulant, moderate-high titers of IgG or IgM anticardiolipin or anti-beta-2-glycoprotein I antibodies, or both, is necessary for the diagnosis of APLS in addition to the presence of clinical criteria. To exclude clinically insignificant or temporary antibodies, the criteria further stipulate that a subsequent APLA test must turn out positive 12 weeks following the first positive test. The diagnosis of APLS is dubious if that period is shorter than 12 weeks or if more than 5 years pass between two distinct clinical symptoms and positive laboratory testing.

In APLS, anemia or thrombocytopenia is often observed. Renal involvement with thrombotic microangiopathy may be indicated by renal failure and proteinuria. The acute thrombotic event may be accompanied by a high erythrocyte sedimentation rate. The rest of the time, inflammation-related indicators are often normal. The presence of these antibodies alone does not establish a diagnosis of SLE in individuals without any clinical signs of SLE.

It is noteworthy that positive ANA and even anti-Ds-DNA are commonly detected in primary APLS without concomitant SLE. Testing for additional hypercoagulable conditions, such as hyperhomocysteinemia, Factor V Leiden and prothrombin variants, shortage of protein C, protein S, or antithrombin III, may be necessary for a patient with numerous thrombotic episodes or pregnancy losses.

Antiphospholipid Syndrome: Treatment

Initial therapy for blood clots often includes a mix of blood-thinning drugs. Heparin and warfarin are the two most widely used. Heparin is injected and has a quick onset of action. Warfarin is a medication that is taken orally and takes several days to work. Additionally a blood thinner, aspirin.

You run a higher risk of bleeding episodes if you use blood thinners. To ensure that your blood can clot sufficiently to stop the bleeding from a cut or the bleeding under the skin from a bruise, your doctor will monitor your dose using blood tests. Some research suggests that additional medications may be effective in treating the antiphospholipid syndrome. These include statins, rituximab, and hydroxychloroquine.

Pregnant patients' care If you get therapy, having an antiphospholipid syndrome pregnancy is still possible. Heparin or heparin plus aspirin is typically used as a treatment. Because warfarin can harm the fetus, pregnant women are not allowed to take it.

Antiphospholipid Syndrome: Prevention

Clinicians can choose the best primary and secondary preventive approach by weighing the thrombotic risk in these patients against the anticipated bleeding risk associated with antithrombotic medication.

Aspirin is advised for first-line prophylaxis in people with SLE, APL, and high-risk profiles who do not have SLE, particularly if other risk factors are present.

In individuals with APS, secondary thrombosis prevention with long-term anticoagulation is generally advised, however in those patients who have bleeding issues, thrombotic risk assessment can help in deciding if continuous anticoagulation is necessary.

Conclusion

Patients with APL are more likely to experience thrombosis, although the severity of the risk depends on several variables, including the APL profile and the existence of other risk factors. Other established predictors of recurrence are also taken into account when determining risk in individuals with thrombosis who fit the requirements for APS.

Dr. Durgesh Kumar Sinha
Dr. Durgesh Kumar Sinha

MBBS MS [ OPHTHALMOLOGY ]

Updated on: 02-Mar-2023

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