Lu Zhaohui, Li Jian, Ba Jianming, Yang Guoqing, Dou Jingtao, Mu Yiming, Lu Juming
Department of Endocrinology, Chinese PLA General Hospital, Beijing, P. R. China.
Neuro Endocrinol Lett. 2009;30(6):700-4.
Lymphocytic hypophysitis (LH) is a rare autoimmune disorder associated with lymphocytic cell infiltration of the pituitary. It is often detected in patients with other autoimmune systemic diseases such as Hashimoto's thyroiditis, epinephritis, megaloblastic anemia, and Langerhans cell histiocytosis. Pituitary biopsy and invasive histopathological examination provide the most reliable evidence for LH diagnosis. However, because glucocorticoids are extensively used for the treatment of disease, pathological diagnosis is not always necessary. Here, we report a rare case of a male patient (50 y) with LH associated with dacryoadenitis that was successfully treated with high-dose of methylprednisolone.
The patient had a history of swollen lacrimal glands for two years. In addition, magnetic resonance imaging (MRI) showed a markedly thickened infundibular stem and an indiscernible signal in the posterior lobe of the pituitary. Biopsy of the lacrimal glands revealed reactive hyperplasia of the lymphoid tissue. After a high-dose of methylprednisolone pulse treatment (HDMPT), lacrimal gland swelling and infundibular stem thickness were reduced, and adenopituitary function improved within a short time period.
Although association of LH with dacryoadenitis is a rare, it can be effectively managed with HDMPT. In addition, response to HDMPT can avoid the more invasive diagnostic procedures, including surgical intervention and pituitary biopsies.
淋巴细胞性垂体炎(LH)是一种罕见的自身免疫性疾病,与垂体淋巴细胞浸润有关。它常出现在患有其他自身免疫性全身性疾病的患者中,如桥本甲状腺炎、肾炎、巨幼细胞贫血和朗格汉斯细胞组织细胞增多症。垂体活检和侵入性组织病理学检查为LH诊断提供最可靠的证据。然而,由于糖皮质激素广泛用于疾病治疗,病理诊断并非总是必要的。在此,我们报告一例罕见的50岁男性LH患者,其伴有泪腺炎,经大剂量甲泼尼龙成功治疗。
该患者有两年泪腺肿大病史。此外,磁共振成像(MRI)显示漏斗柄明显增厚,垂体后叶信号不清。泪腺活检显示淋巴组织反应性增生。经大剂量甲泼尼龙脉冲治疗(HDMPT)后,泪腺肿大和漏斗柄厚度减轻,腺垂体功能在短时间内得到改善。
虽然LH与泪腺炎的关联罕见,但HDMPT可有效治疗。此外,对HDMPT的反应可避免包括手术干预和垂体活检在内的更具侵入性的诊断程序。