IgG4-Related Hypophysitis: A New Addition to the Hypophysitis Spectrum
IgG4-Related Hypophysitis: A New Addition to the Hypophysitis Spectrum
复制标题
DOI：
10.1210/jc.2010-2970
复制
发表时间：
2011-07-01
影响因子：
5.8
通讯作者：
Caturegli, Patrizio
中科院分区：
文献类型：
--
作者：
Leporati, Paola;Landek-Salgado, Melissa A.;Caturegli, Patrizio
背景：脑垂体炎是一种脑下垂体的慢性炎症，包括越来越复杂的临床病理图谱。目的：本研究的目的是描述1例新的IgG4相关性垂体炎患者，回顾已发表的文献，并提供诊断标准。背景：1例75岁男性患者，有1年的额叶头痛病史。初步研究显示全垂体功能减退症，鞍区和蝶窦均有肿块。患者接受经蝶窦手术，开始大剂量泼尼松治疗，然后进行激素替代治疗，并密切监测3年。结果：泼尼松治疗后症状改善，垂体和蝶骨肿块缩小，但当泼尼松剂量减少时，症状复发。组织病理学检查显示，垂体和蝶骨标本中均有明显的单核细胞浸润，主要特征是浆细胞数量增多。许多浸润性浆细胞(每高倍视野10个)呈IgG4阳性。文献复习发现11例IgG4相关性垂体炎(其中两例根据垂体组织病理学诊断)。结论：我们描述了首例经活检证实为IgG4相关性垂体炎的高加索患者，并提供了该疾病的分类标准。(临床内分泌代谢酶96：1971-1980,2011)
Context: Hypophysitis is a chronic inflammation of the pituitary gland that comprises an increasingly complex clinicopathological spectrum. Within this spectrum, lymphocytic and granulomatous hypophysitis are the most common forms, but newer variants have recently been reported.Objective: The aims of the study were to describe a new patient with IgG4-related hypophysitis, review the published literature, and provide diagnostic criteria.Setting: A 75-yr-old man presented with a 1-yr history of frontal headache. Initial studies revealed panhypopituitarism and a mass in both the sella turcica and the sphenoidal sinus. The patient underwent transphenoidal surgery, initiated high-dose prednisone followed by hormone replacement therapy, and was closely monitored for 3 yr.Results: Symptoms improved after prednisone, along with shrinkage of the pituitary and sphenoidal masses, but recurred when prednisone dose was lowered. Histopathology showed a marked mononuclear infiltrate in both the pituitary and sphenoidal specimens, mainly characterized by increased numbers of plasma cells. Many of the infiltrating plasma cells (> 10 per high-power field) were IgG4-positive. Review of the literature identified 11 cases of IgG4-related hypophysitis (two diagnosed based on pituitary histopathology).Conclusions: We describe the first Caucasian patient with biopsy-proven IgG4-related hypophysitis and provide classification criteria for this disease. (J Clin Endocrinol Metab 96: 1971-1980, 2011)