Clinical, immunological features and relation to clinical phenotypes in patients with antiphospholipid syndrome

Aim of the work

To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a cohort of patients and to evaluate the possible clinical-immunologic association of individual antiphospholipid antibodies (aPL) with clinical phenotypes.

Patients and methods

The clinical and immunologic features of APS in 70 patients (17 with primary and 53 with secondary APS) were analyzed. The association between the different aPL and APS clinical phenotypes (venous thrombosis, arterial thrombosis, obstetric morbidity, thrombocytopenia, livedo reticularis) was investigated.

Results

The APS patients were 63 (90%) females, with a mean age of 28.6±6.9 years and a mean disease duration of 3.1±2.6 years. Of the females only 55 were pregnant. Obstetric morbidity was the most common clinical manifestation (87.2%), particularly late fetal death (54.6%), followed by deep venous thrombosis (48.6%), constitutional manifestations (48.6%), thrombocytopenia (24.3%), stroke (15.7%) and livedo reticularis (15.7%). Anticardiolipin (ACL) antibodies were the most common aPL detected (82.9 %). Patients with primary APS had significantly more frequent late abortion/stillbirth (76.5% vs. 44.7%, p= 0.03) compared to secondary APS patients. Lupus anticoagulant (LA) and anti-β2 glycoprotein-I antibodies were significantly associated with arterial thrombosis (p= 0.009 and p< 0.001, respectively), thrombocytopenia (p= 0.01 and p= 0.02, respectively) and livedo reticularis (p= 0.02 and p< 0.001, respectively), while only LA was significantly associated with obstetric complications (p= 0.004). Triple aPL positivity was significantly associated with arterial thrombotic events (p< 0.001) and obstetric complications (p= 0.01).

Conclusion

Patients with APS demonstrated a high frequency of pregnancy complications. Detection of aPL may help in the identification of different clinical phenotypes in APS.