Pediatric Antiphospholipid Antibody Syndrome Follow-up

Updated: Dec 11, 2018
  • Author: Barry L Myones, MD; Chief Editor: Lawrence K Jung, MD  more...
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Follow-up

Further Outpatient Care

Interval care includes the following:

  • History and physical examination for signs and symptoms of thrombotic or vasospastic events

  • Laboratory testing for monitoring anticoagulant therapy, antiphospholipid antibody testing, and, in the case of secondary antiphospholipid syndrome, underlying disease activity

  • Imaging and Doppler studies for follow-up of previous thrombotic process

  • Dietary and lifestyle counseling

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Further Inpatient Care

Further inpatient care in patients with antiphospholipid (aPL) antibody syndrome (APS) is only on an as-needed basis for management of thrombotic events but may include the following:

  • Imaging studies

  • Medical therapy

  • Invasive procedures for thrombolytic therapy

Further inpatient care is indicated if a catastrophic antiphospholipid antibody syndrome (CAPS) occurs.

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Inpatient & Outpatient Medications

See the list below:

  • Antiplatelet therapy, such as administration of aspirin, dipyridamole, hydroxychloroquine, ticlopidine, clopidogrel, or combinations of these agents

  • Vasodilator and/or antiplatelet therapy, such as pentoxifylline or cilostazol

  • Vasodilators, such as niacin or topical nitroglycerin (nitropaste)

  • Anticoagulation therapy, such as warfarin, heparin, or low molecular weight (LMW) heparin [99, 100, 101]

    • Warfarin sensitivity is conferred by the presence of a cytochrome oxidase P-450 mutation (CYP2C9) and can be associated with severe bleeding (*3 isoleucine to leucine in 10% of Caucasians; *4 asparagine to glutamine in 3% of African Americans).

    • The presence of an antiphospholipid antibody accentuates the prothrombotic state that exists when warfarin is withdrawn (because of low protein C synthesis and the presence of plasminogen activator inhibitors).

      • Abrupt withdrawal of warfarin by the physician or by the patient through noncompliance may result in a thrombotic event.

      • Coverage with LMW heparin during the period of warfarin withdrawal (approximately 3-5 d until protein C levels return to normal) may reduce this risk.

    • The prothrombin time (PT), standard partial thromboplastin time, or both may be prolonged in the presence of an antiphospholipid antibody, thus diminishing the accuracy of these assays in monitoring of the effectiveness of anticoagulant therapy.

    • The PT/international normalized ratio (INR) assays are also inaccurate in the presence of the lupus anticoagulant (LAC) and may provide results that vary according to the source of thromboplastin (manufacturer or lot-to-lot).

    • Chromogenic factor X levels and the prothrombin–proconvertin time more accurately reflect the level of anticoagulation in patients with a LAC who are on warfarin therapy.

    • The adequacy of therapy with LMW heparin should be assessed with a plasma anti–factor Xa assay, which measures the inactivation of factor Xa. Ideally, the sample should be drawn 3 hours post-injection, kept at 4°C, and processed as soon as possible.

    • Antiphospholipid antibody can bind to platelet factor 4 (PF4), and/or β2-glycoprotein I [GPI]) in the enzyme-linked immunosorbent assay (ELISA) test for heparin antibodies. A false-positive test in the presence of antiphospholipid antibody–associated thrombocytopenia may be falsely interpreted as heparin-induced thrombocytopenia (HIT), and anticoagulation may be inappropriately halted. Confusion may be avoided by obtaining this test prior to exposure to heparin. [102]

  • Immunomodulators, such as intravenous immunoglobulin or rituximab

  • Therapy for thrombocytopenia, such as steroids, danazol, dapsone, intravenous immunoglobulin (IVIG), or vincristine

  • Therapy for pulmonary hypertension: This may include epoprostenol (prostacyclin PGI2), bosentan (non-selective oral endothelin receptor antagonist), sildenafil (phosphodiesterase-5 inhibitor) [86, 87, 88, 103]

  • Therapy for dyslipoproteinemia or hyperlipidemia: This may include statin drugs or niacin. [95]

  • Dietary supplementation with folic acid, vitamin B-12, or both for patients with hyperhomocysteinemia: See Medication and the therapeutic algorithm in the image below.

    One set of suggested algorithms for the workup and One set of suggested algorithms for the workup and treatment of patients with antiphospholipid antibody syndrome. This should not be considered dogmatic because laboratory evaluation is not standardized and treatment remains empiric and controversial. Laboratory testing is not recommended in healthy asymptomatic individuals with no risk factors and a negative family history.
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Transfer

Patients with CNS, cardiovascular, or peripheral vaso-occlusive events may need to be transferred to facilities with appropriate support personnel, experience, and equipment.

Patients with catastrophic antiphospholipid antibody syndrome require admission to an ICU, high-level supportive care, and multiple consultative services.

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Deterrence/Prevention

See the list below:

  • Adequate medical therapy

  • Patient education

  • Monitoring for new events

  • Monitoring for drug adverse effects and toxicity

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Complications

Hemorrhage may occur as a result of overaggressive therapy.

Rethrombosis may occur as a result of inadequate therapy.

Catastrophic antiphospholipid antibody syndrome can lead to death (50% mortality rate).

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Prognosis

The long-term prognosis varies and depends on the tissue damage incurred and the organ system or systems affected. Clinical manifestations that are associated with a worse prognosis include the following: [104, 105, 106]

  • Pulmonary hypertension

  • Neurologic involvement (eg, CNS involvement, transverse myelopathy)

  • Myocardial ischemia

  • Nephropathy

  • Gangrene of the extremities

  • Catastrophic antiphospholipid antibody syndrome

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Patient Education

Lifestyle counseling is indicated to educate patients and their families about the risk factors that are known to complicate the prognosis of patients with antiphospholipid antibody syndrome.

  • Dietary manipulation is recommended to prevent obesity, hyperlipidemia, and hypertension, starting at a young age, especially in patients with a family history of these problems.

  • Dietary manipulation is recommended to decrease consumption of methionine-containing foods that might increase homocysteine levels in patients carrying mutations of the gene that encodes for methylene tetrahydrofolate reductase mutation (MTHFR). Folate deficiencies need to be identified and corrected in these patients to control homocysteine levels.

  • Counsel adolescents about the potential risks of smoking tobacco in this setting. Provide smoking cessation programs for patients who already have started smoking.

  • In patients with a secondary antiphospholipid antibody syndrome, encourage compliance with medications for control of underlying disease processes, such as vasculitis and systemic lupus erythematosus (SLE).

  • Dietary counseling is indicated for patients on oral anticoagulants.

    • Maintenance of a consistent diet of foods containing vitamin K

    • Avoidance of foods and herbs with anticoagulant properties

  • Counsel patients regarding the risks of oral contraceptive use and the need for alternative methods of contraception.

Current information for patients and their families can be obtained from the excellent Web sites from the St. Thomas Hospital in London (Hughes Syndrome, Hughes Syndrome Foundation).

For excellent patient education resources, visit eMedicineHealth's Brain and Nervous System Center. Also, see eMedicineHealth's patient education articles Blood Clot in the Legs and Stroke.

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