白質病変，肥厚性硬膜炎を呈したWegener肉芽腫症と考えられた1例

A Suspected Case of Wegener Granulomatosis Accompanied with Pachymeningitis and White Matter Lesions

要旨　症例は53歳女性。2003年1月より39℃の発熱が出現。胸部X線で，右上肺野に結節影および左中肺野に空洞を伴う腫瘤影を指摘され，精査目的にて入院した。入院時，WBC 9,500/μl，CRP 13.8mg/dlと炎症所見を認め，肺化膿症を疑いCPFXを投与したところ痙攣が出現し，髄液にて細胞数増加および蛋白の上昇を認めた。頭部CTでは異常を認めず，無菌性髄膜炎と診断し，各種抗微生物薬を投与したが，発熱・頭痛が悪化し，頭部MRIにて脳内病変を認めた。再検査したPR3-ANCAは15 U/mlと上昇し，副鼻腔炎および臨床経過よりWegener肉芽腫症と診断した。PSL 60mg/日，CPA 100mg/日の投与を開始したところ臨床症状・画像所見は改善したものの，その後の造影MRIにて両前頭部の肥厚性硬膜炎を認めた。

　Wegener肉芽腫症に脳内病変・肥厚性硬膜炎を認めた報告は少なく，興味ある1例と思われ報告する。

A 53-year-old woman was admitted to our hospital because of high fever and abnormal chest radiograph shadows. Chest X-ray on admission showed a nodular shadow in the right upper lung field and a mass shadow with a cavity in the left middle lung field. Laboratory data indicated leukocytosis and elevation of C-reactive protein. Pulmonary suppuration was suspected, panipenem/betamipron was prescribed, but a mass and consolidation developed,and the medication was changed to ciprofloxacin. Convulsive seizures with loss of consciousness appeared after the change to ciprofloxacin. Lumbar puncture revealed pleocytosis with a predominance of mononuclear cells（198/3） and elevated protein（83mg/dl）. Brain CT showed no abnormal image, and acute aseptic meningitis was diagnosed and was treated with cefotaxime, clindamycin, fluconazole, acicrovir and sulfamethoxazole/trimethoprim. However, the treatment did not result in symptomatic improvement, and brain MRI showed intracranial disorders. Serum PR3-ANCA was elevated to 15 U/ml. Taken together with chest X-ray, sinusitis and clinical course, a generalized form of Wegener's granulomatosis was diagnosed. She was given 60mg/day of prednisolone, 100mg/day of cyclophosphamide and 9g/day of sulfamethoxazole-trimethoprim and progressively improved. In this process, enhanced MR images showed thickened dural enhancement of the falx and bilateral anterior regions, which showed improvement on brain MRI at 8 months after starting treatment.

　We report a rare case of Wegener's granulomatosis accompanied with pachymeningitis and white matter lesions.

（Received : August 26,2004）