Alba Marco A, Kermani Tanaz A, Unizony Sebastian, Murgia Giuseppe, Prieto-González Sergio, Salvarani Carlo, Matteson Eric L
Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Hospital Universitari Mútua Terrassa, Terrassa, Spain.
Division of Rheumatology, University of California Los Angeles, Los Angeles, CA, USA.
Autoimmun Rev. 2024 Jun;23(6):103580. doi: 10.1016/j.autrev.2024.103580. Epub 2024 Jul 23.
Giant cell arteritis (GCA), the most common primary vasculitis in adults, is a granulomatous systemic vasculitis usually affecting the aorta and its major branches, particularly the carotid and vertebral arteries. Although remission can be achieved in most patients with GCA using high-dose glucocorticoids (GC), relapses are frequent, occurring in >40% of GC-only treated patients, mostly during the first two years after diagnosis. Relapsing courses lead to high GC exposure, increasing the risk of treatment-related adverse effects. Although tocilizumab is an efficacious GC-sparing therapy that allows increased sustained remission and reduced cumulative GC doses, relapses are common after drug discontinuation. This narrative review examines the most relevant features of relapses in GCA, including its definition, classification, frequency, clinical, laboratory, and imaging characteristics, chronology, probable pathophysiology, and predictive factors. In addition, we discuss treatment options for relapsing patients and the effect of relapses on patient outcomes.
巨细胞动脉炎(GCA)是成人中最常见的原发性血管炎,是一种肉芽肿性全身性血管炎,通常累及主动脉及其主要分支,尤其是颈动脉和椎动脉。尽管大多数GCA患者使用大剂量糖皮质激素(GC)可实现缓解,但复发很常见,在仅接受GC治疗的患者中,超过40%会复发,大多发生在诊断后的头两年内。复发过程导致高剂量GC暴露,增加了治疗相关不良反应的风险。尽管托珠单抗是一种有效的糖皮质激素节省疗法,可提高持续缓解率并减少累积GC剂量,但停药后复发很常见。这篇叙述性综述探讨了GCA复发的最相关特征,包括其定义、分类、频率、临床、实验室和影像学特征、时间顺序、可能的病理生理学和预测因素。此外,我们讨论了复发患者的治疗选择以及复发对患者预后的影响。