Lupus Anticoagulant Syndrome

What is Lupus Anticoagulant Syndrome?

Alternatively known as antiphospholipid antibody syndrome, lupus anticoagulant syndrome is a coagulation disorder which causes formation of thrombosis in blood vessels. This condition is attributed to the autoimmune antibody production against a particular phospholipid known as cardiolipin and β2 glycoprotein I. Naturally, these antibodies attack foreign invaders, but when these begin to harm the phospholipid-binding proteins, the blood coagulates unusually.


The syndrome is considered primary when this happens in the nonexistence of other associated disease. More commonly, the disease is secondary as it occurs in conjunction with an autoimmune disorder, systemic lupus erythematosus for instance. In very uncommon circumstances, lupus anticoagulant syndrome causes a person to develop rapid organ failure as a result of generalized thrombosis. This stage of condition is referred as catastrophic antiphospholipid syndrome.

Symptoms

The patient may experience the following signs and symptoms:

  • Arterial and venous thrombosis which can affect any organ system most frequently, deep vein thrombosis in the lower limbs
  • Stroke is the most common arterial event in individuals with this disorder.
  • Patients may suffer from pulmonary embolism. The patient complains of shortness of breath, chest pain ang hemoptysis.
  • A lace-like rash which is purplish in color also known as livedo reticularis appear in the wrist and lower extremities of some affected individuals.
  • Although less common, when thrombosis obstructs the circulation of blood in the brain, the patient may exhibit some neurological manifestations including chronic headaches and migraines, dementia and seizure.
  • Ocular changes
  • Miscarriage which is likely to become recurring may occur before the 20th week of conception in pregnant women affected by the syndrome.
  • Complications of pregnancy including premature birth and preeclampsia
  • People with the disorder may also suffer from cardiovascular problems. The heart valves may become involved. Normally, valves open and close in order to maintain the flow of blood via the heart chambers in only one way. In lupus anticoagulant syndrome, there is affectation of the mitral valve, masses may form on this area or may thicken, leading to blood regurgitation.
  • Low platelet count
  • Patient may show episodes of bleeding, especially from the nose and gums. Petechiae may also appear, suggesting bleeding into the skin.
  • On the odd occasion, a patient can show evidence of movement disorder, cognitive problems, sudden loss of hearing, and depression.

Causes

Phospholipids are fat molecules that contain phosphorus which are typically found all over the body, specifically in living cells and cell membranes. Once a patient develops the disease, the immune system releases antibodies against phospholipids.

Normally, clotting is a basic body process. It assists in closing up minute cuts on the walls of the blood vessels, avoiding too much blood loss. In lupus anticoagulant syndrome, on the other hand, there are unnecessary thrombi forming in the arteries and veins, blocking the circulation of blood, oxygen and nutrients to the organs and tissues.

While the disease was first documented to be present in individuals with systemic lupus erythematosus, at this time, it is understood that majority of these patients do not have the latter.

The main etiology is not wholly determined. Yet, it is known to reduce the concentration of annexin V, a protein attaching to phospholipids which also has a strong anticoagulant activity. Its decline is assumed to be the potential factor which enhances the predisposition of blood to thicken. Anticardiolipin in addition is related to a drop of prostacyclin concentration, which is essential in inhibiting the clumping together of platelets.

Treatment

Treatment is directed at stopping clot and thrombus formation. The condition is regularly treated by administering a combination of blood thinning drugs. Classically, the patient receives a shot of heparin combined with Coumadin. Warfarin is used to inhibit clotting factor synthesis. After a few days of receiving combined drugs, the patient will only receive warfarin as heparin is discontinued. In some instances, aspirin is added to the treatment regimen in order to inhibit platelet activation thus preventing intra-vessel clotting. Treatment involves daily administration of low dose of aspirin 75 mg.

Treatment for pregnant mothers is predominantly complicated as this is costly, involves repeated injections and incorporates a number of adverse reactions. For pregnant women, the pharmacologic treatment comprises of low molecular weight heparin and aspirin in low dose as a replacement for warfarin as this drug tends to be teratogenic as it can cross the placenta. If woman experiences repeated miscarriage, aspirin and low molecular weight heparin such as Lovenox and Fragmin are recommended to be taken after missing a menstrual cycle.

Furthermore, if aspirin is properly complied and heparin shot is administered, a pregnant woman’s chance of a successful delivery can be improved from 10% up to 80%. Eventhough heparin is not recognized to cross the placental barrier; still it is important that one is taking a low molecular form of the drug as prolonged use can lead to osteoporosis.

Lupus Anticoagulant Syndrome and Pregnancy

An expectant mother yet with lupus anticoagulant syndrome can still have successful pregnancies. If the condition is properly managed, these women are expected to bear their infants to term. Management for this group always involves the use of heparin and low dose aspirin. A mother may also need to have periodic sonograms to monitor the fetal growth as babies of individuals with the condition are at an elevated risk for a slowed growth in the uterus.

Is Lupus Anticoagulant Syndrome Hereditary?

This question is still being addressed by researches and scientific studies. Studies suggest that a genetic predisposition of the disease is possible but is not directly hereditary. More than being hereditary, it is likely to be a genetic combination. In addition, these studies imply that the involved antibodies may occur in the family with or without clinical substantiation of antiphospholipid antibody syndrome.

Lupus Anticoagulant Syndrome and Miscarriage

In addition lupus anticoagulant syndrome is the primary reason for roughly 1 out of 5 cases of repeated miscarriage. This is due to the interruption of blood circulation in the blood vessels of the placenta. While the precise series of events is not transparent up till now, it is accurate to say that when the mother is unsupplied with adequate nutrients, the placenta breaks down, and the growing fetus dies.

When miscarriage happens during late trimester in pregnancy, the reason could be strongly associated to lupus anticoagulant syndrome. Not only does this process disrupt blood flow to the placenta, but it is also associated to hypertension, abruptio placenta and limited intrauterine growth. Sadly, a number of women may even experience 6 or more miscarriages ahead of the establishment of the diagnosis and the application of the appropriate medical care.

Diagnosis

Hematological examination, though not thoroughly dependable can still be a useful guide for clinical analysis when combined with the all the information gathered under patient’s history. This examination can detect the presence of anticardiolipin and B2 glycoprotein I antibodies and is conclusive in roughly 80% of the cases of lupus anticoagulant syndrome. Nevertheless, blood tests need to be repetitive as infections and certain drugs can cause antiphospholipid antibodies to be present in the blood for a moment leading to a false positive result. A second blood test may be needed to confirm the result which is performed after about 3 months from the first blood test.

A second analysis, known as lupus anticoagulant test can assist in proving the clinical finding in approximately 40% of cases.

In order to classify the patient’s condition as a case of APS, no less than one of the following conditions must be present;

  • There are lupus anticoagulants in the blood for two or more blood tests which are conducted no less than 3 months apart. Abnormal finding on this antibody suggest a very high chance of developing thrombosis.
  • Average to high concentration of anticardiolipin in the plasma for two or more blood tests which are conducted no less than 3 months apart.
  • Occurrence of mild to extreme concentrations of anti–beta-2 glycoprotein I antibodies on two or more instances at least twelve weeks apart.

Surgery

In refractory situations, pharmacological treatment may not be enough and may already require plasmapheresis. When deep vein thrombosis becomes chronic, an inferior vena cava filter is essentially placed.

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