Department of Endocrinology and Nephrology, The University of Tokyo School of Medicine, Tokyo, Japan.
Endocr J. 2010;57(6):485-92. doi: 10.1507/endocrj.k09e-356. Epub 2010 Apr 1.
A 70-year old man with a 14 year history of Sjögren syndrome, interstitial pneumonia, and autoimmune hepatitis (AIH) was admitted to our hospital due to hyponatremia with a one month history of fatigue, thirst, and nausea. Laboratory tests on admission revealed that this patient had a central adrenal insufficiency. Pituitary magnetic resonance imaging (MRI) further showed swelling of the stalk and posterior lobe of his pituitary, suggesting infundibulo-hypophysitis. Based on his past history of autoimmune disease, his serum IgG4 levels were measured and found to be remarkably high (924 mg/ dL). Previous biopsy specimens from his liver, lung, and parotid gland were immunostained for IgG4, which revealed a marked infiltration of IgG4-positive plasma cells. As a result of our tests, we made a diagnosis of IgG4-related systemic disease. Interestingly, a subsequent MRI scan at three weeks after the patient commenced glucocorticoid replacement therapy for adrenal insufficiency showed that the swelling of his pituitary stalk was reduced. This finding suggested that IgG4-related hypophysitis may improve either as a result of a supplemental dose of glucocorticoid or possibly spontaneously. Although six cases of IgG4-related hypophysitis have been reported in the scientific literature published in English, our current case is the first in which IgG4-related hypophysitis likely occurred as a result of a long-term history of IgG4-related systemic disease. We report this case herein and review the relevant literature.
一位 70 岁男性，患有干燥综合征 14 年、间质性肺炎和自身免疫性肝炎（AIH），因低钠血症伴疲劳、口渴和恶心病史 1 个月而入院。入院时的实验室检查发现该患者存在中枢肾上腺功能不全。垂体磁共振成像（MRI）进一步显示其垂体柄和后叶肿胀，提示漏斗部-垂体炎。基于他过去的自身免疫性疾病史，测量了他的血清 IgG4 水平，结果发现明显升高（924mg/dL）。先前取自他的肝、肺和腮腺的活检标本免疫染色 IgG4，结果显示 IgG4 阳性浆细胞明显浸润。根据我们的检查结果，我们诊断为 IgG4 相关全身性疾病。有趣的是，患者开始接受肾上腺皮质功能不全的糖皮质激素替代治疗 3 周后的后续 MRI 扫描显示，其垂体柄肿胀减轻。这一发现表明 IgG4 相关垂体炎可能由于糖皮质激素的补充剂量或可能自发改善。尽管在已发表的英文科学文献中已报道了 6 例 IgG4 相关垂体炎，但我们目前的病例是首例可能由于长期 IgG4 相关全身性疾病而发生的 IgG4 相关垂体炎。我们在此报告该病例，并复习相关文献。
