Division of Rheumatic Diseases, UT Southwestern Medical Center, Dallas, TX, USA.
Department of Radiology, UT Southwestern Medical Center, Dallas, TX, USA.
Clin Exp Rheumatol. 2019 Mar-Apr;37 Suppl 117(2):144-147. Epub 2018 Sep 20.
Large-vessel inflammation, although rare, has been increasingly recognised as a complication of granulomatosis with polyangiitis (GPA) in recent years. The presentation is highly variable, ranging from an incidental finding to aortic dissection and rupture. Treatment has predominately consisted of a combination of cyclophosphamide and high dose corticosteroids with surgical intervention when indicated. We present the case of a 34-year-old male diagnosed with GPA after presenting with sinus and eye inflammation and the ensuing investigation revealed large vessel involvement that remarkably improved after 6 months of treatment with the combination of rituximab infusions, methotrexate and corticosteroids.
近年来,大血管炎虽然罕见,但已越来越多地被认为是肉芽肿伴多血管炎(GPA)的一种并发症。其表现形式多种多样,从偶发发现到主动脉夹层和破裂不等。治疗主要包括环磷酰胺和大剂量皮质类固醇的联合治疗,并在必要时进行手术干预。我们报告了一例 34 岁男性,因鼻窦和眼部炎症就诊后被诊断为 GPA,随后的检查显示大血管受累,经过 6 个月的利妥昔单抗输注、甲氨蝶呤和皮质类固醇联合治疗后显著改善。