关键词: Connective tissue disease Interstitial lung disease Thrombosis Venous thromboembolism

来  源:   DOI:10.1186/s12959-024-00599-3   PDF(Pubmed)

Abstract:
As an autoimmune disease, the persistent systemic inflammatory response associated with connective tissue disease (CTD) is involved in the development of venous thromboembolism (VTE). However, clinical data showed that the risk of VTE in patients differed between subtypes of CTD, suggesting that different subtypes may have independent mechanisms to promote the development of VTE, but the specific mechanism lacks sufficient research at present. The development of pulmonary fibrosis also contributes to the development of VTE, and therefore, patients with CTD-associated interstitial lung disease (CTD-ILD) may be at higher risk of VTE than patients with CTD alone or patients with ILD alone. In addition, the activation of the coagulation cascade response will drive further progression of the patient\'s pre-existing pulmonary fibrosis, which will continue to increase the patient\'s risk of VTE and adversely affect prognosis. Currently, the treatment for CTD-ILD is mainly immunosuppressive and antirheumatic therapy, such as the use of glucocorticoids and janus kinase-inhibitors (JAKis), but, paradoxically, these drugs are also involved in the formation of patients\' coagulation tendency, making the clinical treatment of CTD-ILD patients with a higher risk of developing VTE challenging. In this article, we review the potential risk factors and related mechanisms for the development of VTE in CTD-ILD patients to provide a reference for clinical treatment and prevention.
摘要:
作为一种自身免疫性疾病,与结缔组织病(CTD)相关的持续性全身炎症反应参与静脉血栓栓塞(VTE)的发生.然而,临床数据显示,CTD亚型之间患者的VTE风险不同,这表明不同的亚型可能有独立的机制来促进VTE的发展,但具体机制目前缺乏足够的研究。肺纤维化的发展也有助于VTE的发展,因此,CTD相关间质性肺病(CTD-ILD)患者发生VTE的风险可能高于单纯CTD患者或单纯ILD患者.此外,凝血级联反应的激活将驱动患者预先存在的肺纤维化的进一步进展,这将继续增加患者的VTE风险并对预后产生不利影响。目前,CTD-ILD的治疗主要是免疫抑制和抗风湿治疗,例如使用糖皮质激素和Janus激酶抑制剂(JAKis),但是,矛盾的是,这些药物也参与了患者凝血倾向的形成,CTD-ILD患者的临床治疗具有较高的VTE发展风险。在这篇文章中,本文对CTD-ILD患者发生VTE的潜在危险因素及相关机制进行综述,为临床治疗和预防提供参考。
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