Anticardiolipin and anti-beta2 glycoprotein I antibodies and lupus-like anticoagulant: prevalence and significance in systemic sclerosis

Br J Dermatol. 2008 Jan;158(1):141-4. doi: 10.1111/j.1365-2133.2007.08309.x. Epub 2007 Nov 19.


Background: It has been suggested that both anticardiolipin (aCL) and anti-beta(2) glycoprotein I (abeta(2)GPI) antibodies may play a critical role in the pathogenesis of systemic sclerosis (SSc)-related vascular impairment.

Objectives: To evaluate the prevalence of aCL and abeta(2)GPI antibodies and lupus-like anticoagulant (LAC) in patients with SSc and healthy controls. We also investigated a possible relationship between clinical and biological variables of patients with SSc and the presence of aCL/abeta(2)GPI antibodies and/or LAC.

Methods: Measurements of aCL and abeta(2)GPI antibodies, and LAC were performed in 69 consecutive patients with SSc and 69 age- and sex-matched controls. Clinical and biological findings were compared between patients with and without antiphospholipid antibodies.

Results: aCL and abeta(2)GPI antibodies and/or LAC were detected in 13 (19%) of 69 consecutive patients with SSc; in the healthy control group, aCL antibody was found in only one (2%) subject (P = 0.0007). None of the healthy controls had abeta(2)GPI antibody and/or LAC. Moreover, pitting scars, pulmonary arterial hypertension, macrovascular involvement as well as severity of capillary impairment (using nailfold videocapillaroscopy) were more frequent in SSc patients with aCL/abeta(2)GPI antibodies and/or LAC compared with those without.

Conclusions: Our findings suggest that antiphospholipid antibodies may have a role in the genesis of vascular involvement related to SSc. Finally, the assessment of antiphospholipid antibodies (aCL and abeta(2)GPI antibodies, as well as LAC) may contribute to a better recognition of clinical features in patients with SSc; in essence, the patients with aCL/abeta(2)GPI antibodies and/or LAC may require close monitoring of vascular changes, including in particular pulmonary arterial hypertension and digital infarcts.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antibodies, Anticardiolipin / blood
  • Antibodies, Antiphospholipid / blood*
  • Female
  • Fetal Death / etiology
  • Fetal Death / immunology
  • Fingers / pathology
  • Humans
  • Lupus Coagulation Inhibitor / blood
  • Male
  • Middle Aged
  • Pregnancy
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / immunology*
  • Scleroderma, Systemic / pathology
  • Skin Ulcer / etiology
  • Skin Ulcer / immunology
  • Skin Ulcer / pathology
  • Venous Thrombosis / etiology
  • Venous Thrombosis / immunology
  • beta 2-Glycoprotein I / immunology


  • Antibodies, Anticardiolipin
  • Antibodies, Antiphospholipid
  • Lupus Coagulation Inhibitor
  • beta 2-Glycoprotein I