Division of Rheumatology Louisiana State University, 1542 Tulane Ave., Box T4M-2, New Orleans, LA, 70112, USA.
Curr Rheumatol Rep. 2018 Apr 2;20(5):25. doi: 10.1007/s11926-018-0737-1.
Giant cell arteritis (GCA) is the most common systemic vasculitis. GCA is categorized as a granulomatous vasculitis of large and medium size vessels. Majority of the symptoms and signs of GCA result from involvement of the aorta and its branches intra- and extracranial. Temporal artery biopsy continues to be the cardinal diagnostic procedure despite new imaging modalities for diagnosing GCA with cranial involvement. Great advances in awareness have led to improvement in preventing irreversible vision loss due to early diagnosis.
The cause of GCA has not been elucidated but major progress has been made in the knowledge of its pathogenesis leading to new therapeutic targets, particularly inhibition of interleukin 6. IL 6 plays a key role in the regulation of TH17/Tregs imbalance in GCA and appears to correlate with clinical disease activity in GCA. All of this has led to the first FDA (food and drug administration) approved treatment for GCA, Tocilizumab. Abatacept and Ustekinumab are promising targets for therapy in LVV but still need further research. This paper is a review of the recent progress in the understanding of GCA pathogenesis, diagnosis, treatment, and prognosis.
巨细胞动脉炎(GCA)是最常见的系统性血管炎。GCA 被归类为大、中血管的肉芽肿性血管炎。GCA 的大多数症状和体征源于颅内外主动脉及其分支受累。尽管有新的成像方式可用于诊断颅内 GCA,但颞动脉活检仍然是主要的诊断程序。由于早期诊断,人们对该病的认识有了很大的提高,从而改善了因不可逆性视力丧失而导致的失明预防。
GCA 的病因尚未阐明,但在其发病机制方面取得了重大进展,从而产生了新的治疗靶点,特别是白细胞介素 6(IL-6)的抑制。IL-6 在 GCA 中 TH17/Tregs 失衡的调节中起着关键作用,似乎与 GCA 的临床疾病活动相关。所有这些都导致了首个获得 FDA(美国食品药品监督管理局)批准用于 GCA 治疗的药物——托珠单抗。阿巴西普和乌司奴单抗是治疗大动脉炎的有前途的靶点,但仍需要进一步研究。本文综述了 GCA 发病机制、诊断、治疗和预后方面的最新研究进展。