Antiphospholipid syndrome: clinical and immunologic manifestation and patterns of disease expression in a cohort of 100 patients / 中华风湿病学杂志

ABSTRACT

Objective To analyze the clinical and immunological manifestations of antiphospholipid syndrome(APS)in a cohort of 100 patients.Methods The clinical and serologic features of APS(Sapporo preliminary criteria)in 100 patients were analyzed retrospectively.Results The cohort consisted of 79 female patients and 21 male patients witb a mean age of 36?13 years at diagnosis.Primary APS was presented in 37% of patients;APS was secondary to systemic lupus erythematosus(SLE)in 46%,lupus-like syndrome in 14%. Eighty percent of the patients had thrombosis,43(54%)patients had venous thrombosis,18(22%)had arterial thrombosis,15(19%)had both arterial and venous thrombosis.4(5%)had thrombosis of microcirculation. Forty-two(52%)patients presented thrombosis at a single site,26(32%)at two sites,12(15%)at three or more sites.Forty-five(56%)patients experienced one thrombotic episode,20(25%)patients had only one re- currence,and 15(19%)had more thrombosis.The most common manifestations of thrombosis were deep vein thrombosis(36%),pulmonary embolism(30%)and stroke(26%),with heart,kidney,gastrointestinal tract and other organs involvements.Thirty-four(51%)female patients had spontaneous fetal losses including intrauter- ine fetus death and recurrent spontaneous abortion.Seventy-one(71%)patients developed thromboeytopenia. The presence of antieardiolipin antibody(ACL)was detected in 84 patients(84%).Among 90 patients with APS,alone ACL was detected in 38 patients(42%),both ACL and lupus anticoagulant(LA)were detected in 36(40%),LA alone in 16(18%).Patients with APS associated with SLE or lupus-like syndrome had higher frequency of arthritis,leukopenia,antinuclear antibodies(ANA)and low complement levels.Female patients had a higher frequency of leukopenia,ANA and ACL.Male patients had a higher prevalence of deep venous thrombosis in the lower limbs and LA.Conclusion APS is an autoimmune disorder characterized by recurrent arterial and venous thrombosis,fetal loss,or thrombocytopenia with the presence of ACL and/or LA.In APS secondary to with SLE,the patient's sex can modify the disease expression and define specific subsets of APS.