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少见的IgG4相关性垂体炎
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Journal Article
少见的IgG4相关性垂体炎
2014
Overview
研究背景 IgG4相关性疾病为新近定义的自身免疫性疾病类型,主要临床特征为多器官受累和血清IgG4水平升高。可发生于垂体,且部分患者表现为孤立性鞍区肿物和（或）垂体柄增粗,由于缺乏特征性影像学表现,若无血清IgG4水平异常之证据,术前明确诊断困难且易误诊为垂体腺瘤。方法与结果男性患者,47岁,临床主要表现为四肢乏力、性功能减退;血清睾酮、皮质醇、卵泡刺激素和黄体生成素均低于正常值范围。头部MRI显示鞍内、鞍上巨大肿物,增强后病灶均匀强化。临床拟诊为垂体腺瘤,手术大部分切除。光学显微镜观察肿物主要为腺垂体,腺泡萎缩、数目减少,间质内可见大量淋巴浆细胞浸润,伴广泛性纤维组织增生、玻璃样变,未见明确的垂体腺瘤结构;边缘可见多灶性合体样细胞巢团,但胞核异型性不明显。免疫组织化学染色,淋巴浆细胞并非肿瘤性单克隆性增生,但IgG4阳性浆细胞数目〉30个/高倍视野且IgG4＋/IgG＋〉40%。合体样细胞巢团波形蛋白、上皮膜抗原和孕激素受体表达阳性;血清IgG4为2.93g/L。明确诊断为IgG4相关性垂体炎伴鞍区脑膜反应。术后予泼尼松35 mg/d持续治疗2周,减至30 mg/d维持治疗,临床症状明显改善,血清IgG4降至正常水平,鞍区肿物体积明显缩小。结论 IgG4相关性垂体炎缺乏特征性影像学表现,其组织学与非特异性炎症性病变相似,术前明确诊断和鉴别诊断困难。血清IgG4水平升高是明确诊断的重要线索和依据。
