Objectives: Analyze the clinical characteristics of patients with primary antiphospholipid syndrome (PAPS) progressing to systemic lupus erythematosus (SLE).Explore the risk factors for the progression from PAPS to SLE. Methods: The clinical data of 262 patients with PAPS enrolled in Peking Union Medical College Hospital from February 2005 to September 2021 were evaluated. Assessments included demographic data, clinical manifestations, laboratory tests (serum levels of complement, anti-nuclear antibodies, anti-double-stranded DNA antibodies), treatment, and outcomes. Kaplan-Meier analysis was used to calculate the prevalence of SLE in patients with PAPS. Univariate Cox regression analysis was employed to identify the risk factors for PAPS progressing to SLE. Results: Among 262 patients with PAPS, 249 had PAPS (PAPS group) and 13 progressed to SLE (5.0%) (PAPS-SLE group). Univariate Cox regression analysis indicated that cardiac valve disease (HR=6.360), positive anti-double-stranded DNA antibodies (HR=7.203), low level of complement C3 (HR=25.715), and low level of complement C4 (HR=10.466) were risk factors for the progression of PAPS to SLE, whereas arterial thrombotic events (HR=0.109) were protective factors (P<0.05 for all). Kaplan-Meier analysis showed that the prevalence of SLE in patients suffering from PAPS with a disease course>10 years was 9%-15%. Hydroxychloroquine treatment had no effect on the occurrence of SLE in patients with PAPS (HR=0.753, 95%CI 0.231-2.450, P=0.638). Patients with≥2 risk factors had a significantly higher prevalence of SLE compared with those with no or one risk factor (13-year cumulative prevalence of SLE 48.7% vs. 0 vs. 6.2%, P<0.001 for both). Conclusions: PAPS may progress to SLE in some patients. Early onset, cardiac-valve disease, positive anti-dsDNA antibody, and low levels of complement are risk factors for the progression of PAPS to SLE (especially in patients with≥2 risk factors). Whether application of hydroxychloroquine can delay this transition has yet to be demonstrated.
目的： 分析原发性抗磷脂综合征（PAPS）进展为系统性红斑狼疮（SLE）患者的临床资料特征，探讨PAPS进展为SLE的危险因素。 方法： 分析2005年2月—2021年9月北京协和医院收治的262例PAPS患者的临床资料，包括人口统计学资料、临床表现、实验室检查（血清补体、抗核抗体、抗双链DNA抗体）、治疗和预后。Kaplan-Meier分析PAPS患者SLE的发生率。单因素Cox回归分析PAPS发生SLE的危险因素。 结果： 262例PAPS患者中，PAPS未发生SLE患者249例（PAPS组），发生SLE患者13例（5.0%）（PAPS-SLE组）。单因素Cox回归分析显示，存在心脏瓣膜病变（HR=6.360）、抗双链DNA抗体阳性（HR=7.203）、低补体C3血症（HR=25.715）、低补体C4血症（HR=10.466）为PAPS发生SLE的危险因素，发生动脉血栓事件（HR=0.109）为保护因素，均P<0.05。Kaplan-Meier分析显示，病程10年以上PAPS患者SLE发生率为9%~15%。羟氯喹治疗对PASP患者是否发生SLE无影响（HR=0.753，95%CI 0.231~2.450，P=0.638）。具有两个及以上危险因素较无或1个危险因素的PAPS患者SLE发生率显著升高（13年SLE累计发生率48.7% 比0 比6.2%，均P<0.001）。 结论： 部分PAPS患者可进展为SLE，早发、存在心脏瓣膜病变、抗双链DNA抗体阳性、低补体血症是PAPS进展为SLE的危险因素，尤其应警惕存在两个及以上危险因素的PAPS患者。羟氯喹应用能否延缓PAPS进展为SLE有待进一步研究。.