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Antiphospholipid antibody syndrome
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Antiphospholipid antibody syndrome

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

Synopsis

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:

Clinical:
  • 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.
Laboratory:
  • 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.

Codes

ICD10CM:
D68.61 – Antiphospholipid syndrome

SNOMEDCT:
26843008 – Antiphospholipid syndrome

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 06/09/2016
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Antiphospholipid antibody syndrome : Cyanosis, Peripheral leg edema, Thrombocytopenia
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