Immune-mediated thrombotic thrombocytopenic purpura with systemic lupus erythematosus: clinical features and outcome.

IF 3.5 2区 医学 Q1 RHEUMATOLOGY
Júlia Weisinger, Raïda Bouzid, Jehane Fadlallah, François Provôt, Pascale Poullin, Véronique Le Guern, David Ribes, Nihal Martis, Yahsou Delmas, Sylvain Chantepie, Virginie Rieu, Ygal Benhamou, Gabriel Choukroun, Manon Marie, Ranta Dana, Agnès Veyradier, Bérangère S Joly, Paul Coppo
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Abstract

Objective: The association of immune-mediated thrombotic thrombocytopenic purpura (iTTP) and SLE was previously described, but patients with iTTP with coexistent SLE remain poorly characterised.

Methods: We compared the clinical presentation and the outcome of patients with iTTP with coexistent SLE (SLE-iTTP) to an age-sex matched cohort of patients with idiopathic iTTP without SLE.

Results: During the study period, 1409 patients with iTTP were recruited in our registry. Of these, 79 (6%) had a prior or concurrent diagnosis of SLE at the time of iTTP diagnosis, and 437 (31%) had detectable ANAs without other clinical features of SLE. When compared with idiopathic iTTP, patients with SLE-iTTP had more severe renal involvement, and cardiac involvement was more prevalent, whereas central nervous system involvement was less common. Patients with SLE-iTTP received more immunosuppressive agents. There was no difference in response categories during the acute phase. During follow-up, SLE-iTTP had superior ADAMTS13 relapse-free survival than idiopathic iTTP. Among patients with ANAs without other clinical features of SLE, 33 (8%) were diagnosed with SLE 27 months (IQR: 7-65 months) following iTTP diagnosis; 32 additional patients (7%) developed another systemic autoimmune disease. No patient from the idiopathic iTTP group developed clinical SLE during follow-up.

Conclusion: Patients with iTTP are prone to develop autoimmune features, and patients with SLE-iTTP have distinct clinical features and outcome. Relapse-free survival seems better in patients with SLE-iTTP, underscoring the need for tailored management strategies in this population, including a specific follow-up to assess early features suggestive of SLE.

Trial registration number: NCT00426686.

Abstract Image

免疫介导的血栓性血小板减少性紫癜合并系统性红斑狼疮:临床特征和结果。
目的:免疫介导的血栓性血小板减少性紫癜(iTTP)与SLE之间的关联此前已有报道,但iTTP合并SLE患者的特征仍然很差。方法:我们将iTTP合并SLE (SLE-iTTP)患者的临床表现和结果与年龄性别匹配的特发性iTTP无SLE患者进行比较。结果:在研究期间,我们的登记处招募了1409名iTTP患者。其中,79例(6%)在iTTP诊断时有SLE的先前或同时诊断,437例(31%)有可检测到的ANAs,但没有SLE的其他临床特征。与特发性iTTP相比,SLE-iTTP患者有更严重的肾脏受累,心脏受累更普遍,而中枢神经系统受累则不常见。SLE-iTTP患者使用更多的免疫抑制剂。急性期两组患者的反应类型无差异。随访期间,slei -iTTP患者的ADAMTS13无复发生存率优于特发性iTTP患者。在没有其他SLE临床特征的ANAs患者中,33例(8%)在iTTP诊断后27个月(IQR: 7-65个月)被诊断为SLE;另外32名患者(7%)发展为另一种系统性自身免疫性疾病。在随访期间,特发性iTTP组无患者发生临床SLE。结论:iTTP患者易出现自身免疫特征,SLE-iTTP患者具有明显的临床特征和转归。SLE- ittp患者的无复发生存率似乎更好,这强调了在这一人群中需要量身定制的管理策略,包括评估提示SLE的早期特征的特定随访。试验注册号:NCT00426686。
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来源期刊
Lupus Science & Medicine
Lupus Science & Medicine RHEUMATOLOGY-
CiteScore
5.30
自引率
7.70%
发文量
88
审稿时长
15 weeks
期刊介绍: Lupus Science & Medicine is a global, peer reviewed, open access online journal that provides a central point for publication of basic, clinical, translational, and epidemiological studies of all aspects of lupus and related diseases. It is the first lupus-specific open access journal in the world and was developed in response to the need for a barrier-free forum for publication of groundbreaking studies in lupus. The journal publishes research on lupus from fields including, but not limited to: rheumatology, dermatology, nephrology, immunology, pediatrics, cardiology, hepatology, pulmonology, obstetrics and gynecology, and psychiatry.
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