Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
Mod Rheumatol Case Rep. 2023 Jun 19;7(2):453-457. doi: 10.1093/mrcr/rxad009.
Giant cell arteritis (GCA) is a large vessel vasculitis that primarily involves aorta and its major branches. Cerebral infarction is a serious complication that can occur secondary to GCA in up to 3% of patients with a mortality rate of over 50%. Due to the rarity of this severe complication, no therapeutic strategies are currently available. Furthermore, despite the recent progress in molecular-targeted therapy for GCA, it remains unknown whether tocilizumab is effective for severe ischemic complications such as cerebral infarction. The accumulation of individual cases in which this fatal complication could be treated is apparently required to build a better management of the disease. We present our case of GCA that developed severe cerebral infarction during high-dose glucocorticoid and tocilizumab therapy, and its symptoms and image findings were improved by switching to intravenous cyclophosphamide. Our case suggests that an intensive immunosuppressive therapy, including cyclophosphamide, may be necessary to stabilise this fatal complication of GCA.
巨细胞动脉炎(GCA)是一种主要累及主动脉及其主要分支的大血管血管炎。脑梗死是一种严重的并发症,在高达 3%的 GCA 患者中可继发出现,死亡率超过 50%。由于这种严重并发症较为罕见,目前尚无治疗策略。此外,尽管 GCA 的分子靶向治疗最近取得了进展,但尚不清楚托珠单抗是否对脑梗死等严重缺血性并发症有效。显然需要积累个别病例,以更好地管理这种疾病。我们报告了一例 GCA 患者,在大剂量糖皮质激素和托珠单抗治疗期间发生严重脑梗死,通过改用静脉注射环磷酰胺,其症状和影像学发现得到改善。我们的病例提示,包括环磷酰胺在内的强化免疫抑制治疗可能是稳定 GCA 这种致命并发症所必需的。