Ida M, Iwata M, Horiguchi T, Oda S, Takeuchi E, Nakamura Y, Satou A
Department of Respiratory Medicine, Haibara General Hospital, Shizuoka, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1996 Nov;34(11):1283-8.
A 54-year-old man was referred to our hospital because of fever, abdominal pain, hematemesis, and dyspnea on exertion. Laboratory examination on admission revealed elevations of WBC count and CRP. a low serum complement level, hypoxemia, and microhematuria. A chest X-ray film and a CT scan showed bilateral interstitial pneumonia and cardiomegaly. Angiography showed microaneurysms in peripheral branches of the superior mesenteric artery, the hepatic artery, and the right renal artery. Examination of a specimen of the right quadriceps muscle revealed arteritis with fibrinoid necrosis in a small artery, which was consistent with polyarteritis nodosa (PN). A specimen obtained by transbronchial lung biopsy before treatment showed interstitial mononuclear cell infiltration and edema. PN with interstitial pneumonia was diagnosed and the patient was treated with 1 g of methylprednisolone for 3 days, followed by 60 mg of prednisolone and 100 mg of cyclophosphamide daily. The interstitial pneumonia progressed. Despite a second trial of steroid pulse therapy in addition to mechanical ventilation and various kinds of antibiotics, the patient died of respiratory failure on the 35th hospital day. A specimen obtained by transbronchial lung biopsy 3 days before death revealed proliferation of fibroblasts, mononuclear cell infiltration in the interstitium, and hyaline membrane formation in air spaces. No evidence of infection was found. Interstitial pneumonia associated with PN had been considered to be quite rare, but recently some cases resembling the present case have been reported. We believe that interstitial pneumonia associated with PN might not be rare, and the present case shows that it can be fulminant.
一名54岁男性因发热、腹痛、呕血和劳力性呼吸困难被转诊至我院。入院时实验室检查显示白细胞计数和C反应蛋白升高、血清补体水平降低、低氧血症和镜下血尿。胸部X线片和CT扫描显示双侧间质性肺炎和心脏增大。血管造影显示肠系膜上动脉、肝动脉和右肾动脉的外周分支有微动脉瘤。右股四头肌标本检查显示一条小动脉有伴有纤维蛋白样坏死的动脉炎，这与结节性多动脉炎（PN）相符。治疗前经支气管肺活检获得的标本显示间质单核细胞浸润和水肿。诊断为PN合并间质性肺炎，患者接受1g甲泼尼龙治疗3天，随后每日给予60mg泼尼松龙和100mg环磷酰胺。间质性肺炎进展。尽管除机械通气和各种抗生素外还进行了第二次类固醇冲击治疗试验，患者仍在住院第35天死于呼吸衰竭。死亡前3天经支气管肺活检获得的标本显示成纤维细胞增生、间质单核细胞浸润和气腔内透明膜形成。未发现感染证据。PN相关的间质性肺炎曾被认为非常罕见，但最近有一些与本病例相似的病例报道。我们认为PN相关的间质性肺炎可能并不罕见，本病例表明其可能呈暴发性。
