A suspected case of Wegener granulomatosis accompanied with pachymeningitis and white matter lesions]

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(83 mg/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 60 mg/day of prednisolone, 100 mg/day of cyclophosphamide and 9 g/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.