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Antiphospholipid antibody syndrome
Other Resources UpToDate PubMed

Antiphospholipid antibody syndrome

Contributors: Vivian Wong MD, PhD, Susan Burgin MD, Art Papier MD, William Van Stoecker MD
Other Resources UpToDate PubMed


The antiphospholipid antibody syndrome (APLS) is an acquired autoimmune disease characterized by the formation of autoantibodies, which include the anti-cardiolipin antibody, lupus anticoagulant, and anti-beta-2-glycoprotein I antibody. APLS is commonly due to an underlying autoimmune disease such as systemic lupus erythematosus (SLE). Less frequently, an underlying infection (including human immunodeficiency virus or hepatitis C) or a malignancy (such as lymphoproliferative disease) is seen. Primary APLS can occur. In addition, several medications are associated with APLS, including chlorpromazine, hydralazine, and procainamide.

APLS is defined by the presence of elevated titers of anti-phospholipid antibody with concomitant recurrent episodes of arterial, venous, small vessel thrombosis, and/or obstetric morbidity. Aside from painful cutaneous ulcers and necrosis, symptoms are variable depending on the organ system involved. The most common thrombotic events occur in the deep venous system, usually in the leg. Obstetric complications include premature delivery, unexplained fetal loss beyond 10 weeks of gestation, or 3 or more episodes of unexplained consecutive spontaneous abortions before 10 weeks of gestation. Respiratory compromise may signal a pulmonary embolism. A long list of neurologic deficits due to cerebral events has been compiled, including severe migraine headaches, visual disturbances, and stroke. Renal, cardiac, hepatic, adrenal, and gastrointestinal thromboses produce their own symptomatology. Some of these patients have the clinical manifestations of Sneddon syndrome. In rare cases, catastrophic antiphospholipid antibody syndrome with rapid development of widespread thrombotic disease and multi-organ involvement can develop, which is associated with a poor prognosis.

The Sapporo criteria for diagnosis of APLS were developed by an international expert panel in 1999 and revised in 2006. According to the revised criteria, one clinical finding along with one positive laboratory criterion must be present. While the 2006 criteria have been noted by some to be less sensitive than the 1999 criteria, their diagnostic and laboratory parameters are still useful in establishing a diagnosis:

  • An episode of arterial or venous thrombosis that is confirmed with an imaging study, or an episode of small vessel thrombosis.
  • Unexplained spontaneous abortion of a morphologically normal fetus after 10 weeks of pregnancy.
  • A premature birth of an otherwise healthy infant before 34 weeks of pregnancy due to maternal complications such as preeclampsia, eclampsia, or placental insufficiency.
  • Two or more positive tests for anticardiolipin antibody (IgG and/or IgM) in serum or plasma at least 12 weeks apart by enzyme-linked immunosorbent assay (ELISA).
  • Two or more positive plasma tests for lupus anticoagulant at least 12 weeks apart.
  • Two or more positive tests for and anti-beta-2-glycoprotein I antibody (IgG and/or IgM) in serum or plasma at least 12 weeks apart by ELISA.


D68.61 – Antiphospholipid syndrome

26843008 – Antiphospholipid syndrome

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Last Updated:06/08/2016
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Antiphospholipid antibody syndrome
A medical illustration showing key findings of Antiphospholipid antibody syndrome : Cyanosis, Peripheral leg edema, Thrombocytopenia
Clinical image of Antiphospholipid antibody syndrome - imageId=166082. Click to open in gallery.  caption: 'Large, deep, granulating, slough-covered ulcers with surrounding hyperpigmentation and erythema on the leg.'
Large, deep, granulating, slough-covered ulcers with surrounding hyperpigmentation and erythema on the leg.
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