Sidney Kimmel Medical College, Thomas Jefferson University Hospital, Philadelphia, PA, USA.
Department of Neurological Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA.
Mod Rheumatol Case Rep. 2021 Jul;5(2):342-346. doi: 10.1080/24725625.2021.1909222. Epub 2021 Apr 15.
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a small- and medium-vessel autoimmune vasculitis. Rare presentations of GPA can manifest as ophthalmologic and endocrinological deficits with sellar enhancement on imaging. While GPA typically presents distinct in appearance from other sellar pathologies, such as pituitary adenoma, we report the case of a 41-year-old woman with GPA of the pituitary that was initially diagnosed as pituitary macroadenoma with apoplexy and treated with two surgical resections without improvement of clinical symptoms. Pathology analysis of the second resection specimen revealed an inflammatory process consistent with GPA. After the pathologic and clinical diagnosis of GPA was established, treatment with steroid and steroid-sparing immunosuppressants resulted in improvements both on imaging and symptomatically. We discuss important aspects of the diagnosis and treatment of this rare presentation of GPA.
肉芽肿性多血管炎（GPA），以前称为韦格纳肉芽肿，是一种小血管和中等血管自身免疫性血管炎。GPA 的罕见表现可表现为眼科学和内分泌学缺陷，并伴有鞍上增强成像。虽然 GPA 通常与其他鞍区病变（如垂体腺瘤）表现明显不同，但我们报告了一例 41 岁女性的垂体 GPA，最初诊断为垂体大腺瘤伴卒中，并接受了两次手术切除，但临床症状没有改善。第二次切除标本的病理分析显示符合 GPA 的炎症过程。在确立 GPA 的病理和临床诊断后，使用类固醇和类固醇保留免疫抑制剂治疗导致影像学和症状均有改善。我们讨论了这种罕见 GPA 表现的诊断和治疗的重要方面。
