González Revilla Elizabeth Manuely, Fernandez Araceli Abad, Ramirez María Teresa Río, Pardo Sara Calero, Moragues María Antonia Juretschke
Hospital Universitario de Getafe, Madrid, Espana.
Respir Med Case Rep. 2016 Aug 26;19:121-4. doi: 10.1016/j.rmcr.2016.08.009. eCollection 2016.
Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the kidney and can very rarely affect large vessels such as the aorta and the surrounding retroperitoneal tissue. Early diagnosis and treatment is of vital importance because of the high risk of dissection and of obstruction of retroperitoneal structures. We present the case of a 74-year-old man with a past history of infrarenal abdominal aortic aneurysm. He consulted for abdominal pain. Cavitating pulmonary nodules and retroperitoneal fibrosis with periaortic alterations were detected on computed tomography. Laboratory investigations revealed that the patient was positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and necrotizing granulomas were observed on biopsies of the lung lesions and retroperitoneal tissue. The patient was diagnosed with GPA and treatment was started with glucocorticoids and immunosuppressive agents, which led to a significant clinical and radiological improvement over the following months.
肉芽肿性多血管炎（GPA）是近年来用于指代韦格纳肉芽肿的名称。这种疾病是一种全身性炎症性疾病，其特征为坏死性血管炎，可累及中小血管（毛细血管、小动脉、小静脉和动脉）。肉芽肿性炎症会影响呼吸系统；通常也会累及肾脏，极少数情况下会影响大血管，如主动脉及周围的腹膜后组织。由于存在夹层分离和腹膜后结构梗阻的高风险，早期诊断和治疗至关重要。我们报告一例74岁男性患者，既往有肾下腹主动脉瘤病史。他因腹痛前来就诊。计算机断层扫描发现有空洞性肺结节以及伴有主动脉周围改变的腹膜后纤维化。实验室检查显示患者的胞浆型抗中性粒细胞胞浆抗体（c-ANCA）呈阳性，在肺部病变和腹膜后组织活检中观察到坏死性肉芽肿。该患者被诊断为GPA，并开始使用糖皮质激素和免疫抑制剂进行治疗，在接下来的几个月里临床和影像学表现有了显著改善。
