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antiphospholipid syndrome

MIM.107320

Monday 25 October 2004

Definition: The antiphospholipid syndrome is characterized by clinical evidence of arterial or venous thrombosis, thrombocytopaenia, recurrent fetal loss and repeated positivity of antiphospholipid autoantibodies.

The designation ’antiphospholipid syndrome’ was proposed for the association of arterial and venous thrombosis, recurrent fetal loss, and immune thrombocytopenia with a spectrum of autoantibodies directed against cellular phospholipid components.

Anticardiolipin antibodies may react with cardiolipin and with other negatively charged phospholipids.

The term ’lupus anticoagulant’ refers to a heterogeneous group of antibodies, most commonly of the IgG type, that are detected by their inhibitory effect on coagulant-active phospholipid components of in vitro coagulation tests.

Synopsis

- recurrent thrombophlebitis
- pulmonary lesions

  • pulmonary thromboembolism
  • pulmonary hypertension
  • microvascular pulmonary thrombosis
  • pulmonary capillaritis
  • alveolar haemorrhage

- adrenal lesions

  • adrenal ischemic necrosis (adrenal ischemia)

- hepatic lesions

  • hepatic ischemia

- renal lesions

  • glomerular capillary thrombi
  • renal afferent arteriolar thrombi

- arterial lesions

  • arterial fibrin thrombi
  • widespread obstruction by recanalized intimal connective tissue
  • concentric cellular and fibrous intimal hyperplasia in renal, leptomeningeal, and pulmonary small arteries (as in hypertensive vascular disease)
  • arterial thrombosis
  • arterial intimal hyperplasia
  • arterial acute inflammation

See also

- thrombosis
- intimal hyperplasia

Laboratory

- antiphospholipid antibodies
- lupus anticoagulant

References

- Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med. 2002 Mar 7;346(10):752-63. PMID: #11882732#

- Hughson MD, McCarty GA, Brumback RA. Spectrum of vascular pathology affecting patients with the antiphospholipid syndrome. Hum Pathol. 1995 Jul;26(7):716-24. PMID: #7628842#