Zhang J T, Qi W T, Zhou Y Z, Huang C, Zhao J L, Li M T, Zeng X F
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730,China.
Zhonghua Nei Ke Za Zhi. 2023 Feb 1;62(2):147-155. doi: 10.3760/cma.j.cn112138-20220429-00325.
We sought to investigate the clinical characteristics and risk factors of antiphospholipid syndrome (APS) complicated by autoimmune hemolytic anemia (AIHA). Retrospective anaysis.Three hundred fifteen consecutive patients with APS were enrolled at the Department of Rheumatology of Peking Union Medical College Hospital between May 2017 to May 2021, and their clinical manifestations[including initial symptoms, time interval between APS onset and diagnosis, systemic lupus erythematosus(SLE), thrombotic events, obstetric morbidity, and extra-criteria manifestations] and laboratory test results[including blood routine, antiphospholipid antibodies(aPLs), blood lipid profile, homocysteine, anti-nuclear antibody profile, immunoglobulin levels, and complement levels] were collected. Then, univariate and multivariate logistic regression analyses were performed. Clinical features and risk factors were analyzed using univariable and multivariable logistic regression analysis. Among 315 APS patients, 37 cases (11.7%) were complicated by AIHA, and AIHA was the first manifestation or co-occurrence. The median time interval between APS onset and diagnosis was 12 months. The proportion of SLE in APS patients combined with AIHA was higher than that in APS patients without AIHA[62.2%(23/37) vs. 19.4%(54/278), <0.001]. There was no significant difference in the proportions of thrombosis and pregnancy morbidity between the two groups. In terms of extra-criteria manifestations, APS patients with AIHA had a significantly (<0.05) greater risk of thrombocytopenia (=6.19, 95% 2.81-13.65) and higher proportions of hypocomplementemia, a positive lupus anticoagulant (LA) result, double aPLs positivity[i.e., any two of the following antibodies were positive: LA, anticardilolipin antibody(aCL), and anti-β2 glycoprotein Ⅰ(β2GPⅠ)], and triple aPLs positivity (i.e., LA, aCL, and anti-β2GPⅠ antibodies were all positive). Multivariate logistic regression analysis showed that SLE (=3.46,95% 1.60-7.48), thrombocytopenia (=2.56,95% 1.15-5.67), and hypocomplementemia (=4.29,95% 2.03-9.04) were independent risk factors for the complication of APS. In the primary APS subgroup, multivariate logistic regression analysis showed that livedo reticularis (=10.51,95%CI 1.06-103.78), thrombocytopenia (=3.77, 95% 1.23-11.57), and hypocomplementemia (=5.92,95% 1.95-17.95) were independent risk factors for the complication of APS. AIHA is not rare in APS patients; moreover, it occurs more frequently in APS secondary to SLE and is more likely to present with a variety of extra-criteria manifestations. Patients with AIHA should be promptly tested for antiphospholipid antibody profiles and alerted to the possibility of thrombotic events.
我们旨在研究抗磷脂综合征(APS)合并自身免疫性溶血性贫血(AIHA)的临床特征及危险因素。回顾性分析。2017年5月至2021年5月期间,北京协和医院风湿免疫科连续纳入315例APS患者,收集其临床表现[包括初始症状、APS发病至诊断的时间间隔、系统性红斑狼疮(SLE)、血栓事件、产科并发症及额外标准表现]和实验室检查结果[包括血常规、抗磷脂抗体(aPLs)、血脂谱、同型半胱氨酸、抗核抗体谱、免疫球蛋白水平及补体水平]。然后进行单因素和多因素逻辑回归分析。采用单变量和多变量逻辑回归分析对临床特征和危险因素进行分析。在315例APS患者中,37例(11.7%)合并AIHA,且AIHA为首发表现或同时出现。APS发病至诊断的中位时间间隔为12个月。APS合并AIHA患者中SLE的比例高于未合并AIHA的APS患者[62.2%(23/37)对19.4%(54/278),<0.001]。两组间血栓形成和妊娠并发症的比例无显著差异。在额外标准表现方面,合并AIHA的APS患者血小板减少风险显著更高(<0.05)(比值比=6.19,95%可信区间2.81 - 13.65),低补体血症、狼疮抗凝物(LA)结果阳性、aPLs双阳性[即以下任何两种抗体阳性:LA、抗心磷脂抗体(aCL)和抗β2糖蛋白Ⅰ(β2GPⅠ)]及aPLs三阳性(即LA、aCL和抗β2GPⅠ抗体均阳性)的比例更高。多因素逻辑回归分析显示,SLE(比值比=3.46,95%可信区间1.60 - 7.48)、血小板减少(比值比=2.56,95%可信区间1.15 - 5.67)和低补体血症(比值比=4.29,95%可信区间2.03 - 9.04)是APS发生并发症的独立危险因素。在原发性APS亚组中,多因素逻辑回归分析显示,网状青斑(比值比=10.51,95%可信区间1.06 - 103.78)、血小板减少(比值比=3.77,95%可信区间1.23 - 11.57)和低补体血症(比值比=5.92,95%可信区间1.95 - 17.95)是APS发生并发症的独立危险因素。AIHA在APS患者中并不罕见;此外,其在继发于SLE的APS中更常见,且更易出现多种额外标准表现。应及时对AIHA患者进行抗磷脂抗体谱检测,并警惕血栓事件的发生。