Ams

Antiphospholipid Syndrome
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Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Blood clots, recurrent miscarriages, rash
Complications	Stroke, heart attack, deep vein thrombosis
Onset
Duration
Types	N/A
Causes	Autoimmune disorder
Risks
Diagnosis	Blood tests for antiphospholipid antibodies
Differential diagnosis	N/A
Prevention	N/A
Treatment	Anticoagulants, immunosuppressants
Medication	N/A
Prognosis	Variable
Frequency
Deaths	N/A

Antiphospholipid Syndrome (APS), also known as Hughes Syndrome, is an autoimmune disorder characterized by the presence of antiphospholipid antibodies in the blood, which increases the risk of blood clots. APS can lead to various complications, including deep vein thrombosis, stroke, and recurrent miscarriages.

Pathophysiology

APS is caused by the immune system mistakenly attacking certain normal proteins in the blood, specifically those that bind to phospholipids. The main antibodies involved are anticardiolipin antibodies, lupus anticoagulant, and anti-β2 glycoprotein I antibodies. These antibodies interfere with the normal function of the coagulation system, leading to an increased risk of clot formation.

Clinical Manifestations

The clinical manifestations of APS can vary widely among patients. Common symptoms and complications include:

• Thrombosis: APS is most commonly associated with venous and arterial thrombosis. Deep vein thrombosis (DVT) and pulmonary embolism are frequent venous complications, while stroke and myocardial infarction are common arterial events.
• Pregnancy-related complications: APS is a significant cause of recurrent miscarriages, stillbirth, and other pregnancy complications such as preeclampsia and intrauterine growth restriction.
• Skin manifestations: Some patients may develop a characteristic rash known as livedo reticularis, which appears as a mottled, purplish discoloration of the skin.
• Neurological symptoms: In addition to stroke, APS can cause migraine, seizures, and cognitive dysfunction.

Diagnosis

The diagnosis of APS is based on clinical criteria and laboratory tests. The Sydney criteria are commonly used, which require at least one clinical event (such as thrombosis or pregnancy morbidity) and the presence of antiphospholipid antibodies on two or more occasions at least 12 weeks apart.

Laboratory Tests

Key laboratory tests for diagnosing APS include:

• Anticardiolipin antibodies: Detected using an ELISA test.
• Lupus anticoagulant: Assessed through a series of coagulation tests, including the dilute Russell's viper venom time (dRVVT).
• Anti-β2 glycoprotein I antibodies: Also measured using an ELISA test.

Treatment

The primary treatment for APS involves the use of anticoagulants to prevent clot formation. The choice of anticoagulant and the duration of treatment depend on the patient's history and risk factors.

• Warfarin: A common oral anticoagulant used for long-term management.
• Heparin: Often used in acute settings or during pregnancy.
• Aspirin: Low-dose aspirin may be used in some cases, particularly for patients with a history of pregnancy complications.

In some cases, immunosuppressants or corticosteroids may be used to manage severe or refractory cases of APS.

Prognosis

The prognosis for patients with APS varies depending on the severity and frequency of thrombotic events and the presence of other autoimmune conditions, such as systemic lupus erythematosus (SLE). With appropriate management, many patients can lead normal lives, although they may require lifelong anticoagulation therapy.

See Also

• Systemic lupus erythematosus
• Thrombophilia
• Autoimmune disease

External Links

• APS Information from the Lupus Foundation