Woywodt A, Knoblauch H, Kettritz R, Schneider W, Göbel U
Department of Nephrology, Franz-Volhard-Clinic, Humboldt-University, Berlin, Germany.
Scand J Rheumatol. 2000;29(4):264-6. doi: 10.1080/030097400750041433.
Involvement of brain parenchyma or meninges in ANCA-associated small-vessel vasculitis such as Wegener's granulomatosis (WG) is not uncommon. In contrast, involvement of the pituitary is exceedingly rare with only a few cases reported so far. The diagnosis is usually made on the basis of imaging techniques and abnormal pituitary function tests in the setting of active systemic vasculitis. However, histology-proven involvement of the pituitary by WG has not been reported so far. We report a case of WG with histology-proven granulomatous necrotizing inflammation of the pituitary and hypothalamo-pituitary stalk, disclosed at autopsy after the patient had died suddenly and unexpectedly in his sleep. In a setting of histology-proven WG, these findings were regarded as a pituitary manifestation of the disorder. A distinct cause of death could not be found, hence we speculate that hypothalamo-pituitary inflammation due to WG may have caused the sudden death in this patient.
在抗中性粒细胞胞浆抗体（ANCA）相关的小血管炎（如韦格纳肉芽肿病，WG）中，脑实质或脑膜受累并不罕见。相比之下，垂体受累极为罕见，迄今为止仅有少数病例报道。诊断通常基于影像学检查以及在活动性系统性血管炎背景下垂体功能测试异常做出。然而，迄今为止尚未有经组织学证实的WG累及垂体的报道。我们报告一例WG患者，经组织学证实垂体及下丘脑 - 垂体柄存在肉芽肿性坏死性炎症，该病例是在患者于睡眠中突然意外死亡后尸检时发现的。在经组织学证实的WG背景下，这些发现被视为该疾病的垂体表现。未发现明确的死因，因此我们推测WG所致的下丘脑 - 垂体炎症可能是该患者猝死的原因。
