Nakajima T, Yasuba H, Yamashita K, Kita H, Sumitomo S, Nakata K, Kato M
Department of Respiratory Medicine, Takatsuki Red Cross Hospital, Osaka, Japan.
Nihon Kokyuki Gakkai Zasshi. 1999 Oct;37(10):807-11.
A 68-year-old woman was admitted to our hospital because of fever of unknown origin and pain in the lower extremities. Six weeks after onset, diffuse infiltrative shadows were observed on chest X-ray films, and marked hypoxemia and progressive renal dysfunction suddenly developed. Corticosteroid therapy (2 courses of pulse therapy, each consisting of methylprednisolone at 500 mg/day for 3 days) was not effective, and the patient died 9 weeks after onset because of respiratory failure. Serologic tests were positive for MPO-ANCA. Histopathologic findings at autopsy disclosed arteriolar fibrinoid necrosis in tissues of the liver, spleen, lungs, and kidneys, thus yielding a diagnosis of microscopic polyangiitis. Lung specimens also demonstrated massive alveolar hemorrhaging in the mid-lung fields and diffuse alveolar damage (DAD) in all lobes. Pulmonary hemorrhage coexistent with DAD worsens the prognosis for microscopic polyangiitis in patients positive for MPO-ANCA.
一名68岁女性因不明原因发热及下肢疼痛入住我院。发病六周后，胸部X线片显示弥漫性浸润阴影，且突然出现明显低氧血症和进行性肾功能不全。皮质类固醇治疗（2个疗程的冲击治疗，每个疗程包括甲基强的松龙500mg/天，共3天）无效，患者在发病9周后因呼吸衰竭死亡。血清学检查MPO-ANCA呈阳性。尸检组织病理学结果显示肝、脾、肺和肾组织的小动脉纤维蛋白样坏死，从而诊断为显微镜下多血管炎。肺标本还显示肺中叶大量肺泡出血及各肺叶弥漫性肺泡损伤（DAD）。MPO-ANCA阳性患者中，与DAD共存的肺出血会使显微镜下多血管炎的预后恶化。
