Anticardiolipin and anti-β2glycoprotein I antibodies and lupus-like anticoagulant: prevalence and significance in systemic sclerosis

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.