| Objective: By means of making a summary about the clinical features,immunologicaltests,treatment measures and outcomes both of SLE-APS and PAPS,the purpose of this studyis to find the similarities and differences between PAPS and SLE-APS so as to strengthen theunderstanding of APS secondary to SLE,and except to give a advise about appropriate earlytherapy to improve the condition of disease.Methods: A retrospective analysis of clinical symptoms, immunological tests, organinvolvements, disease activities, treatments, and outcomes of seven cases with SLE-APS wasmade. We’ve reviewed the case reports published on FMJC database and domestic literaturesabout SLE-APS since1994,and compared the clinical symptoms, immunological tests with alarge sample of PAPS patients reported by literature.Results:In seven cases of patients with secondary APS, the male to female ratio is1:6; twocases with a onset of APS performance, and the rest with a typical onset of SLE followed byAPS in2.43years on overage.The incidences of thrombotic events, morbid history ofpregnancy, thrombocytopenia were71%,66.7%,57%, respectively. All patients’ antibody ofACL were positive(positive rate of100%) and positive rate of aβ2GP1is57%.Four patients’SLEDAI scores≧10when APS events occurred.All patients received glucocorticoidtherapy,6patients received one or more injection of high-dose glucocorticoid shock,cyclophosphamide pulse therapy,gamma-globulin therapy and anticoagulant therapy. Twopatients underwent plasmapheresis treatment. As a result,1patient was discharged, theremaining six cases were improved. Of all English literatures reviewed in this study, the rateof male to female in35cases is1:6.Seventeen patients were diagnosed as SLE firstly，7cases appeared repeated pregnancy failures or thromboembolic events before, and11casesmeet the classification criteria for both SLE and APS at the beginning.The incidences ofthromboembolism, morbid pregnancy and thrombocytopenia were80%,44%and45.7%, respectively, The positive rates of ACL, LA, aβ2GP1were77%,48.6%,25.7%,respectively,61.9%of patients had SLEDAI scores≧10when APS eventsoccurred.We have detailed records of treatment of25patients, they all acceptedglucocorticoid therapy,twenty patients were treated with methylprednisolone pulse therapy,cyclophosphamide pulse, intravenous gamma globulin treatment; plasmapheresis andbiologics application was applied on2patients respectively;10cases accepted oralimmunosuppressants;24patients were treated with anticoagulant therapy. At last21patientswere improved,2patients were discharged,2died. Reviewed in Chinese literatures of25patients with SLE-APS, the clinical features, treatments are similar to English literatures. Acomparison about the clinical manifestations and immunological features was made betweenSLE-APS and PAPS cases, and showed that the majority of both clinical characteristics weresimilar, but livedo reticularis, pulmonary embolism in patients with PAPS were morecommon (24%/6%, P <0.01;36%/18%, P <0.05).On the contrary, when the APS secondaryto SLE, leukopenia was more susceptible (27%/2%, P <0.01). Anti-ds-DNA antibody, ENA,ANA positive, low complement C4hyperlipidemia were more common in patients withSLE-APS (P <0.01), and ACL positive rates had no significant difference (P>0.05).Astatistical analysis of the relationship between initial treatment with the medium-termoutcome pointed out that early anticoagulation, glucocorticoid therapy may prevent thefurther development of disease (P <0.05).Conclusion: SLE-APS usually happened in female patients with SLE at childbearing age andmostly occurred in the lupus’ active phase, it could also occur before or at the same time withSLE, its most clinical features were similar to PAPS.Once diagnosed, early anticoagulation orglucocorticoid therapy is beneficial to control the disease. |
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