Mongera Nicola, Di Maso Vittorio, Ermacora Elisabetta, Carraro Michele, Bregant Cristina, Pian Martina, Savi Umberto, Lorenzon Eric, Boscutti Giuliano
SC Nefrologia e Dialisi-ASUITs, Azienda Sanitaria Universitaria Integrata di Trieste.
G Ital Nefrol. 2018 May;35(3).
Granulomatosis polyangiitis (GPA) is an ANCA-related vasculitis (AAV) whose clinical manifestations mainly concern the respiratory tract (upper and lower) and the kidney. The treatment of GPA (as well as other AAV) includes the use of immunosuppressive drugs with numerous side effects; the most frequent complications are infectious and neoplastic. GPA frequently relapses. Epstein Barr Virus (EBV) is a ubiquitous virus; it is estimated that about 90% of the world’s population has BEEN EXPOSED TO with this pathogen and has subsequently developed a latent infection. Under certain conditions including immunosuppression EBV may reactivate. We report the clinical case of a 67-year-old woman who presented with GPA involving the upper respiratory tract and renal failure with the need for hemodialysis treatment. The fourth month of induction therapy with cyclophosphamide and methylprednisone she presented with dyspnea and respiratory failure. After excluding pulmonary embolism and heart failure, a series of investigations including high resolution tomography and fibroscopy with broncoalveolar lavage (BAL) were performed which excluded recurrence of pulmonary vasculitis including alveolar haemorrhage A BAL demonstrated EBV-DNA. On this basis EBV pneumonia was diagnosed, and antiviral therapy with acyclovir was begun, followed by clinical and radiological improvement. In patients with GPA treated with immunosuppressive drugs pulmonary involvement may not only be due to the underlying vasculitis, but also to opportunistic agents, which must always be considered.
肉芽肿性多血管炎(GPA)是一种与抗中性粒细胞胞浆抗体相关的血管炎(AAV),其临床表现主要涉及呼吸道(上呼吸道和下呼吸道)和肾脏。GPA(以及其他AAV)的治疗包括使用具有众多副作用的免疫抑制药物;最常见的并发症是感染性和肿瘤性并发症。GPA经常复发。爱泼斯坦-巴尔病毒(EBV)是一种普遍存在的病毒;据估计,世界上约90%的人口已接触过这种病原体,并随后发展为潜伏感染。在包括免疫抑制在内的某些条件下,EBV可能会重新激活。我们报告了一例67岁女性的临床病例,该患者患有累及上呼吸道的GPA和需要血液透析治疗的肾衰竭。在使用环磷酰胺和甲基泼尼松进行诱导治疗的第四个月,她出现了呼吸困难和呼吸衰竭。在排除肺栓塞和心力衰竭后,进行了一系列检查,包括高分辨率断层扫描和支气管肺泡灌洗(BAL)纤维镜检查,排除了包括肺泡出血在内的肺血管炎复发。BAL显示EBV-DNA。在此基础上诊断为EBV肺炎,并开始使用阿昔洛韦进行抗病毒治疗,随后临床和影像学症状改善。在接受免疫抑制药物治疗的GPA患者中,肺部受累不仅可能归因于潜在的血管炎,还可能归因于机会性病原体,必须始终予以考虑。