Department of Immunology, Transplantation and Infections Disease, Vita-Salute San Raffaele University and San Raffaele Scientific Institute, Milan, Italy.
Vascular Sciences and Rheumatology, Imperial Centre for Translational and Experimental Medicine, National Heart and Lung Institute, Imperial College London, Hammersmith Hospital, London, UK.
Rheumatology (Oxford). 2019 Feb 1;58(2):206-219. doi: 10.1093/rheumatology/key040.
Although outcomes in Takayasu arteritis (TAK) are improving, diagnosis is typically delayed and significant arterial injury accrues. While wider use of non-invasive imaging is impacting this, the onus remains with clinicians to consider a diagnosis of TAK earlier. Meanwhile, morbidity and mortality in TAK remains increased. Herein we review the current situation, outline recent advances and summarize remaining challenges. Understanding of disease pathogenesis remains poor. However, recent genetic data and identification of pathogenic cytokines may facilitate the search for biomarkers capable of distinguishing active and inactive disease, inflammatory and non-inflammatory arterial remodelling. Imaging is critical for TAK, and each modality has important strengths and limitations. Dependence upon CS therapy remains too high. However, the impact of combination immunosuppressive therapy is now recognized, biologic therapies are increasingly available and new agents offer promise. Multicentre clinical trials are now required, and these will depend upon development of defined clinical and imaging end-points.
虽然 Takayasu 动脉炎（TAK）的治疗效果在不断改善，但诊断通常会延迟，导致严重的动脉损伤。尽管非侵入性影像学的广泛应用正在对此产生影响，但临床医生仍有责任更早地考虑 TAK 的诊断。与此同时，TAK 的发病率和死亡率仍然较高。本文综述了目前的情况，概述了最近的进展，并总结了仍然存在的挑战。目前对疾病发病机制的了解仍不完善。然而，最近的遗传数据和致病细胞因子的鉴定可能有助于寻找能够区分活动期和非活动期疾病、炎症性和非炎症性动脉重塑的生物标志物。影像学检查对 TAK 至关重要，每种影像学检查都有其重要的优势和局限性。对 CS 治疗的依赖仍然过高。然而，联合免疫抑制疗法的影响现已得到认识，生物疗法越来越多，新药物也带来了希望。现在需要进行多中心临床试验，而这将取决于明确的临床和影像学终点的制定。
