Background Antiphospholipid syndrome (APS) is an acquired autoimmune prothrombotic condition characterized by persistent circulating antiphospholipid antibodies (APL). The pathogenic mechanisms that lead to clinical manifestations associated with APL are only partially understood. And to date, long-term anticoagulation has been the only treatment shown to reduce vascular complications.
Objectives The aims of the present study were to assess and identify the prognostic factors of the long-term outcomes and mortality of antiphospholipid syndrome (APS) in Chinese patients.
Methods Records of 160 patients with APS admitted to Peking Union Medical College Hospital in Beijing between 2005 and 2015 were investigated. Demographic characteristics, cumulative clinical and laboratory features, autoantibody profiles were retrieved from the database. Survival rates were studied by Kaplan-Meier method, and COX proportional hazard model was adopted to perform the analysis of predicting factors for mortality.
Results The entire cohort consisted of 110 (68.8%) female and 50 (31.3%) male patients. Mean (SD) age at study entry was 36.5±14.9 years. The most prevalent thrombotic risk factors were hypertension, dyslipidemia, and smoking, present in 5–15% of the total cohort. In total, 50.6% of the patients had primary APS, 45.9% had APS associated with SLE, 2.0% APS associated with other connective tissue diseases. The most prevalent immunological features at baseline were LA (71.3%), aCL (55.0%), and β2GPI (49.4%). No significant statistical differences were found in the clinical presentation of the APS according to the presence or absence of any of these antibodies. During the 10-year period, 16 (10.0%) patients (8 female and 8 male) died. The overall 1, 3, and 5-year survival rate was 92.6%, 89.1% and 87.1%, respectively. The most common causes of death were severe thrombotic events, including pulmonary embolism, strokes and myocardial infarction (43.8% of total deaths), infections (18.8%). COX proportional hazard model show thrombocytopenia is the independent prognostic factor of mortality (HR 8.228, 95% CI 1.866–36.282).
Conclusions patients with APS develop significant morbidity and mortality despite current treatment. More attention should be devoted to APS patients with thrombocytopenia.
Disclosure of Interest None declared