Division of Rheumatology, Department of Medicine, Arcispedale Santa Maria Nuova Istituto di Ricovero e Cura a Carattere Scientifico, Viale Risorgimento, 80, 42123, Reggio Emilia, Italy.
University of Modena and Reggio Emilia, Modena, Italy.
Clin Rev Allergy Immunol. 2018 Apr;54(2):244-260. doi: 10.1007/s12016-017-8643-2.
Despite the progress in the last years on the field of vasculitides, there are several unmet needs regarding classification, disease activity assessment, predictors of flares and complications, and type of treatment for the different forms. The 1990 American College of Rheumatology (ACR) classification criteria currently used to define giant cell arteritis and Takayasu arteritis were designed to discriminate between different types of vasculitides but not to differentiate vasculitis from other disorders. Recently, efforts have been made to overcome the shortcomings of the ACR criteria. The lack of an accepted definition of disease activity in large-vessel vasculitides presents a major challenge in creating useful and valid outcome tools for the assessment of disease course. Identification of predictors of flares can aid in optimizing therapeutic strategies, minimizing disease flares, and reducing treatment-related side effects. It is furthermore important to recognize and characterize the risk factor that might predict the manifestations associated with poor outcome and prognosis. Two RCTs have evidenced the efficacy of tocilizumab in addition to glucocorticoids (GCs) in the treatment of giant cell arteritis (GCA). However, the role of tocilizumab or other biological agents without GCs needs to be investigated. Recent observational studies have suggested that rituximab is also effective in patients with eosinophilic granulomatosis with polyangiitis and in antineutrophil cytoplasmic antibodies (ANCA)-negative patients with granulomatosis with polyangiitis and microscopic polyangiitis. Rituximab or anti-TNF alfa may represent a possible alternative therapy in case of refractory or difficult to treat polyarteritis nodosa (PAN) patients. The new International Criteria for Behçet's Disease have shown a better sensitivity and a better accuracy compared to the older International Study Group on Behçet's Disease criteria. The EULAR recommendations for the management of Behçet's disease (BD) have been recently updated. However, the treatment of refractory disease is still a real challenge.
尽管过去几年在血管炎领域取得了进展，但在分类、疾病活动评估、发作和并发症预测因素以及不同类型的治疗方面仍存在一些未满足的需求。目前用于定义巨细胞动脉炎和 Takayasu 动脉炎的 1990 年美国风湿病学会 (ACR) 分类标准旨在区分不同类型的血管炎，但不能将血管炎与其他疾病区分开来。最近，人们努力克服 ACR 标准的缺点。大动脉血管炎中缺乏公认的疾病活动定义，这在创建用于评估疾病过程的有用和有效的结局工具方面带来了重大挑战。识别发作的预测因素可以帮助优化治疗策略、最大限度地减少疾病发作和减少治疗相关的副作用。此外，识别和描述可能预测与不良结局和预后相关的表现的危险因素也很重要。两项 RCT 证明了托珠单抗联合糖皮质激素 (GCs) 在治疗巨细胞动脉炎 (GCA) 中的疗效。然而，需要研究托珠单抗或其他无 GCs 的生物制剂的作用。最近的观察性研究表明，利妥昔单抗在嗜酸性肉芽肿伴多血管炎和抗中性粒细胞胞浆抗体 (ANCA) 阴性的肉芽肿伴多血管炎和显微镜下多血管炎患者中也有效。在难治性或难以治疗的多动脉炎 (PAN) 患者中，利妥昔单抗或抗 TNF alfa 可能是一种替代治疗方法。与旧的国际 Behçet 病研究组标准相比，新的 Behçet 病国际标准显示出更好的敏感性和准确性。EULAR 最近更新了 Behçet 病 (BD) 的管理建议。然而，难治性疾病的治疗仍然是一个真正的挑战。
