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Central diabetes insipidus as the presenting symptom of granulomatosis with polyangiitis

机译:中枢尿崩症为肉芽肿合并多血管炎的表现

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Granulomatosis with polyangiitis (Wegener's; GPA) is a necrotizing granulomatous vasculitis that can affect any organ system; however, central nervous system involvement is uncommon. We present 2 cases where central diabetes insipidus was the presenting manifestation of GPA. Pituitary involvement was documented by magnetic resonance imaging. Both patients were female and responded to combination therapy of prednisone and cyclophosphamide. We also review the literature for all reported cases of pituitary involvement in GPA. We conclude that pituitary involvement in GPA seems more common in females and may be the initial presenting clinical feature and may occur in the absence of systemic disease. Early recognition and treatment may prevent pituitary necrosis and irreversible damage.
机译:肉芽肿伴多血管炎(韦格纳氏; GPA)是一种坏死性肉芽肿性血管炎,可影响任何器官系统。但是,中枢神经系统受累并不常见。我们目前有2例中枢性尿崩症是GPA的表现。磁共振成像记录了垂体受累。两名患者均为女性,对泼尼松和环磷酰胺的联合治疗有反应。我们还回顾了所有报道的GPA垂体受累病例的文献。我们得出的结论是,垂体受累于GPA在女性中似乎更为常见,并且可能是最初出现的临床特征,并且可能在没有全身性疾病的情况下发生。尽早识别和治疗可能会预防垂体坏死和不可逆的损害。

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