Horne A E, Henriksen P A, Amft E N
AE Horne, Rheumatic Diseases Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK. Email
J R Coll Physicians Edinb. 2014;44(4):283-5. doi: 10.4997/JRCPE.2014.406.
Polyangiitis with granulomatosis, previously known as Wegener's granulomatosis, is a systemic necrotising granulomatous vasculitis. It predominantly affects the upper and lower respiratory tracts and the kidneys, but can potentially affect any organ system. It is diagnosed by clinical features, immunology (anti-neutrophil cytoplasmic antibodies) and histology. Cardiac involvement occurs in 6 to 44% of cases. We present a case of polyangiitis with granulomatosis and constrictive pericarditis, which occurred despite vigorous immunosuppression and which required surgical pericardectomy.
肉芽肿性多血管炎,以前称为韦格纳肉芽肿,是一种全身性坏死性肉芽肿性血管炎。它主要影响上、下呼吸道和肾脏,但也可能影响任何器官系统。其诊断依据临床特征、免疫学(抗中性粒细胞胞浆抗体)和组织学检查。6%至44%的病例会出现心脏受累。我们报告一例肉芽肿性多血管炎合并缩窄性心包炎的病例,尽管进行了积极的免疫抑制治疗仍发生了该病,且需要进行心包切除术。