尿崩症を合併した臨床的にWegener肉芽腫症が示唆された1例

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  • A case of clinically suggested Wegener's granulomatosis complicated by diabetes insipidus.

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Diabetes insipidus is an uncommon complication of Wegener's granulomatosis. We report a case of clinically suggested Wegener's granulomatosis complicated by cranial diabetes insipidus in which evidence of intracranial granuloma extension was found by head CT scan. A 43 year-old female was admitted to our hospital with complaints of nasal obstruction, occular pain, polydipsia and polyuria, with high fever.<br>On admission she had rodent corneal ulcer and blepharoptosis on the right side, saddle nose, skin rash and ulcer. Chest X-ray showed bilateral cavitary pulmonary nodules in the left upper and right middle lobes. Laboratory findings revealed elevated ESR, positive CRP and RA, RAHA, increased serum IgG and serum immune complex. The diagnosis of Wegener's granulomatosis was mainly made by clinical manifestations. The histological examination of biopsied materials from nasal mucosa and lung disclosed necrotizing granulation tissue. A vasopressin test confirmed cranial diabetes insipidus. Head CT scan showed mass shadow in the paranasal sinuses and parasellar region. Therefore, diabetes insipidus may be due to involvement of the hypothalamus or pituitary stalk by granulomatous infiltration.<br>She was successfully treated with combination therapy of predonisolone and cyclophosphamide. After daily intranasal DDAVP was stopped, the diabetes insipidus and Wegener's granulomatosis have both been in remission.

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