Kirchner J, Raab H P, Länger F, Wigand R, Mitrou P, Jacobi V
Institut für Röntgendiagnostik, Klinikum der Johann Wolfgang Goethe-Universität, Frankfurt am Main.
Aktuelle Radiol. 1998 May;8(3):114-8.
Antineutrophil cytoplasmatic antibodies (ANCA)-associated vasculitides (Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome) show quite variable courses. Clinical features of the full blown generalized systemic vasculitis are usually found in the respiratory tract and the kidney. Pulmonary involvement of Wegener's granulomatosis shows commonly nodules and cavitations but also diffuse alveolar hemorrhage. We report the case of a 57 year-old man suffering from dyspnea, thoracal pain, arthralgia, purpura, scleritis and tinitus. Specimen of the kidney showed segmental glomerulosclerosis and tubulointerstitial nephritis. Because of the presence of cANCA Wegener's disease was assumed. Pulmonary infiltrates developed under immunosuppressive treatment with cyclophosphamid. As differential diagnosis of the pulmonary infiltrates, we considered invasive pulmonary aspergillosis as well as infiltrates due to Wegener's granulomatosis. In spite of maximal therapeutic management of patient died of respiratory and cardiovascular failure. The findings at autopsy showed distinct invasive pulmonary aspergillosis and perifocal hemorrhage.
抗中性粒细胞胞浆抗体（ANCA）相关血管炎（韦格纳肉芽肿、显微镜下多血管炎、变应性肉芽肿性血管炎）病程差异较大。典型的全身性系统性血管炎的临床特征通常见于呼吸道和肾脏。韦格纳肉芽肿的肺部受累常见结节和空洞形成，也可出现弥漫性肺泡出血。我们报告一例57岁男性患者，有呼吸困难、胸痛、关节痛、紫癜、巩膜炎和耳鸣症状。肾脏标本显示节段性肾小球硬化和肾小管间质性肾炎。由于存在胞浆型抗中性粒细胞胞浆抗体（cANCA），考虑为韦格纳病。在环磷酰胺免疫抑制治疗过程中出现肺部浸润。对于肺部浸润的鉴别诊断，我们考虑侵袭性肺曲霉病以及韦格纳肉芽肿所致浸润。尽管给予了最大程度的治疗，患者仍死于呼吸和心血管衰竭。尸检结果显示有明显的侵袭性肺曲霉病和病灶周围出血。
