Ma Jian-Min, Li Jing, Ge Xin, Li Ming, Li Jin-Ru, Wang Xiao-Na
Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology and Visual Sciences Key Laboratory, Beijing 100730, China.
Chin Med J (Engl). 2015 Feb 20;128(4):493-8. doi: 10.4103/0366-6999.151102.
Benign lymphoepithelial lesion (BLEL) is characterized by symmetric bilateral swelling of the lacrimal and salivary glands and considered a subtype of immunoglobulin G4 (IgG4)-related sclerosing disease, the etiology and pathogenesis of which has not been determined. The purpose of the present study was to analyze the clinical features of BLEL in the lacrimal gland and the relationship between the serum level of IgG4 and BLEL.
Twenty consecutive patients with BLEL diagnosed in Department of Ophthalmology at Beijing Tongren Hospital, Capital Medical University between January 2012 and December 2013 were observed. The clinical features, imaging findings, laboratory tests, treatments and follow-up status of these 20 consecutive patients were analyzed.
In all 20 patients, the ratio of male to female was 1:4, the ages ranged from 28 to 57 years, the ratio of unilateral to bilateral eyes involvement was 1:4, and painless uncongested symmetrical swelling of the upper eyelid was the main clinical manifestation. Orbital magnetic resonance imaging (MRI) showed that all patients involved lacrimal gland, which were obviously enlarged with equal signals in T1W images and T2W images and obvious enhancement on contrast MRI. Extraocular muscles were involved in 5 patients, salivary gland in 8 patients, and frontal nerve in 3 patients. Serum IgG4 concentration was elevated in 18 patients. The treatment strategy mainly included surgery and steroid administration. Three patients were lost to follow-up, 17 patients reached complete response, and no recurrence was observed.
Eyelid swelling is the typical symptom of BLEL. Most of the patients involved bilateral lacrimal glands. High serum IgG4 level and abundant IgG4-positive plasma cell infiltration are the important features, which can be found in most of BLEL patients. Surgery combined with glucocorticoids is an efficient treatment strategy.
良性淋巴上皮病变（BLEL）的特征为泪腺和唾液腺对称性双侧肿大，被认为是免疫球蛋白G4（IgG4）相关硬化性疾病的一种亚型，其病因和发病机制尚未明确。本研究的目的是分析泪腺BLEL的临床特征以及血清IgG4水平与BLEL之间的关系。
观察2012年1月至2013年12月期间在首都医科大学附属北京同仁医院眼科确诊的20例连续BLEL患者。分析这20例连续患者的临床特征、影像学表现、实验室检查、治疗及随访情况。
20例患者中，男女比例为1:4，年龄范围为28至57岁，单眼与双眼受累比例为1:4，主要临床表现为上睑无痛性、无充血的对称性肿胀。眼眶磁共振成像（MRI）显示所有患者均累及泪腺，泪腺明显肿大，在T1加权像和T2加权像上信号均匀，增强MRI上有明显强化。5例患者累及眼外肌，8例累及唾液腺，3例累及额神经。18例患者血清IgG4浓度升高。治疗策略主要包括手术和使用类固醇。3例患者失访，17例患者达到完全缓解，未观察到复发。
眼睑肿胀是BLEL的典型症状。大多数患者累及双侧泪腺。血清IgG4水平升高和IgG4阳性浆细胞大量浸润是重要特征，在大多数BLEL患者中均可发现。手术联合糖皮质激素是一种有效的治疗策略。
