Clinical, biological, prognostic characteristics of patients with immune-mediated thrombotic thrombocytopenic purpura and Sjögren's disease.

IF 5.1 2区 医学 Q1 RHEUMATOLOGY
Justine Luciano, Laurent Gilardin, Gaétane Nocturne, Raïda Bouzid, Agnès Veyradier, Xavier Mariette, Paul Coppo, Isabelle Bonnet, Bérangère S Joly
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Abstract

Objectives: The association between immune-mediated thrombotic thrombocytopenic purpura (iTTP) and Sjögren disease (SjD) has been poorly investigated. This study presents the first retrospective cohort of iTTP-SjD aiming to identify risk factors for iTTP occurrence in SjD patients and examine their clinical course.

Methods: Patients with iTTP-SjD were identified within the French TTP Registry based on American College of Rheumatology/European League Against Rheumatism 2016 criteria. A comparative analysis was conducted with two control groups comprising primary SjD (pSjD) patients from the French ASSESS cohort and idiopathic iTTP patients from the French TTP Registry. Demographic, clinical and biological data were retrospectively collected.

Results: Thirty iTTP-SjD patients were included and compared with 65 pSjD and 45 idiopathic iTTP patients. The majority of iTTP-SjD patients (n=18) were diagnosed with SjD at the time of iTTP diagnosis. In comparison with the pSjD cohort, iTTP-SjD patients were diagnosed with SjD at a younger age (p=0.039) and showed a higher prevalence of anti-SjS-related antigen A antibody positivity and xerostomia (p=0.015, p=0.035, respectively). EULAR Sjogren's Syndrome Disease Activity Index showed similar activity levels between the two groups. iTTP-SjD patients were treated with plasma exchange (n=28), corticosteroids, rituximab (n=19) and caplacizumab (n=3). In comparison with the idiopathic iTTP cohort, mortality rates (log-rank tests, p=0.228), biological and clinical iTTP relapses (multivariate analysis, p=0.181) were comparable and short-term outcomes (survival at day 30, relapse) were favourable.

Conclusion: iTTP can be a rare complication in patients with SjD. Further studies involving larger cohorts and long-term follow-up are warranted to confirm these findings and to explore the efficacy of immunomodulators and caplacizumab in iTTP-SjD patients.

免疫介导的血栓性血小板减少性紫癜和斯约格伦病患者的临床、生物学和预后特征。
目的:免疫介导的血栓性血小板减少性紫癜(iTTP)与斯约格伦病(SjD)之间的关联性研究很少。本研究首次对 iTTP-SjD 进行了回顾性队列研究,旨在确定 SjD 患者发生 iTTP 的风险因素并研究其临床过程:根据美国风湿病学会/欧洲抗风湿联盟2016年标准,在法国TTP登记处确定了iTTP-SjD患者。与两个对照组进行了比较分析,对照组包括来自法国ASSESS队列的原发性SjD(pSjD)患者和来自法国TTP登记处的特发性iTTP患者。回顾性收集了人口统计学、临床和生物学数据:结果:纳入了 30 名 iTTP-SjD 患者,并与 65 名 pSjD 患者和 45 名特发性 iTTP 患者进行了比较。大多数 iTTP-SjD 患者(18 人)在确诊 iTTP 时就被诊断为 SjD。与 pSjD 患者队列相比,iTTP-SjD 患者确诊 SjD 的年龄更小(p=0.039),抗 SjS 相关抗原 A 抗体阳性率和口腔干燥症发生率更高(分别为 p=0.015 和 p=0.035)。iTTP-SjD患者接受了血浆置换(28人)、皮质类固醇、利妥昔单抗(19人)和卡普珠单抗(3人)治疗。与特发性 iTTP 队列相比,死亡率(对数秩检验,P=0.228)、生物和临床 iTTP 复发率(多变量分析,P=0.181)相当,短期疗效(第 30 天存活率、复发率)良好。结论:iTTP可能是SjD患者的罕见并发症,有必要进行更大规模的研究和长期随访,以证实这些发现,并探索免疫调节剂和卡普拉珠单抗对iTTP-SjD患者的疗效。
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来源期刊
RMD Open
RMD Open RHEUMATOLOGY-
CiteScore
7.30
自引率
6.50%
发文量
205
审稿时长
14 weeks
期刊介绍: RMD Open publishes high quality peer-reviewed original research covering the full spectrum of musculoskeletal disorders, rheumatism and connective tissue diseases, including osteoporosis, spine and rehabilitation. Clinical and epidemiological research, basic and translational medicine, interesting clinical cases, and smaller studies that add to the literature are all considered.
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