Antiphospholipid syndrome history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Feham Tariq, MD [2]

Overview

Antiphosphilipid syndrome can cause (arterial/venous) blood clots (in any organ system) or pregnancy-related complications (especially miscarriages in the second or third trimester). In APS patients, the most common venous event is deep vein thrombosis of the lower extremities (blood clot of the deep veins of the legs). The most common arterial event is a stroke. Patients presenting with antiphosphoplipid syndrome have a positive history of deep venous thrombosis, myocardial infarction and stroke. Last trimester miscarriages, history of heart murmurs or cardiac valvular vegetations and hemolytic anemias may also be present.

History

Patients presenting with antiphosphoplipid syndrome (APS) may have a positive history of:

• Thrombosis (eg, DVT/PE, MI, transient ischemic attack [TIA], or stroke, especially if recurrent, at an earlier age, or in the absence of other known risk factors)
• Miscarriage (especially late trimester or recurrent) or premature birth
• History of heart murmur or cardiac valvular vegetations
• History of hematologic abnormalities, such as thrombocytopenia or hemolytic anemia
• History of nephropathy
• Nonthrombotic neurologic symptoms, such as migraine headaches, chorea, seizures, transverse myelitis, Guillain-Barré syndrome, or dementia (rare)
• Unexplained adrenal insufficiency
• Avascular necrosis of bone in the absence of other risk factors
• Pulmonary hypertension

Common Symptoms

Other common findings, although not part of the APS Classification Criteria, are thrombocytopenia (low platelet count), heart valve disease, and livedo reticularis (a skin condition).

• Associated sign and symptoms may include:

  • Major Features:

    • Venous thrombosis
    • Arterial thrombosis
    • Thrombocytopenia
    • Recurrent fetal loss

  • Minor Features:

    • Migraine
    • Livedo reticularis
    • Endocardial valvular vegetations
    • Transverse myelopathy
    • Chorea
    • Leg ulcers

Less Common Symptoms

Some patients report headaches and migraines. Antiphospholipid syndrome can rarely mimic multiple sclerosis with an estimated 10% of patients misdiagnosed.

Very few patients with primary APS go on to develop SLE.

• Other signs and symptoms include:

  • Myocardial Infarction
  • Stroke
  • Pulmonary embolism
  • Amaurosis fugax
  • Retinal infarct
  • Other visceral infarct
  • Peripheral or deep venous occlusion
  • Raynaud’s syndrome
  • Pulmonary hypertension
  • Avascular necrosis
  • Recurrent fetal loss (especially in the 2nd or 3rd trimester)
  • Pre-eclampsia
  • Adrenal insufficiency
  • Coomb’s positive hemolysis
  • Sudden multi-systemic occlusion (the Catastropic antiphospholipid-antibody syndrome).