Abstract:

Objective To explore the etiology, clinical manifestations, diagnosis and treatment of IgG4-related Mikulicz disease and to provide a reference for clinical diagnosis and treatment. Methods Case data from a patient with IgG4-related Mikulicz disease occurring in the submandibular gland and the literature were retrospectively analyzed. Results IgG4-related Mikulicz disease is a rare autoimmune disease. Its typical features include lacrimal gland and salivary gland swelling, a significant increase in the serum IgG4 level (> 1.35 g/L), and IgG4-positive plasma cell infiltration into a large number of pathological tissues. The disease can easily recur. Treatment is mainly symptomatic, including surgical resection and administration of glucocorticoid and other drug treatment. Long-term follow-up is required after the operation. Conclusion IgG4-related Mikulicz disease has no specific clinical features. The diagnosis relies mainly on pathological examination and serum IgG4 examination. The main treatment methods are surgery and the use of glucocorticoids, which require long-term follow-up.

Key words: IgG4, Mikulicz disease, autoimmune disease, submandibular gland enlargement, tissue fibrosis, glucocorticoids

摘要：

目的 探讨IgG4相关性Mikulicz病的病因、临床表现、诊断及治疗,为临床诊疗提供参考。方法 对1例发生于下颌下腺的IgG4相关性Mikulicz病患者的病例资料及文献进行回顾性分析。结果 IgG4相关性Mikulicz病是一种较为罕见的自身免疫性疾病,其典型特征包括泪腺及唾液腺肿大、血清IgG4水平显著提高（> 1.35 g/L）及病理组织IgG4阳性浆细胞大量浸润。诊断主要依靠病理学检查。治疗以对症治疗为主,包括手术切除、服用糖皮质激素等治疗方法。该病容易复发,术后需要长期随访。结论 IgG4相关性Mikulicz病无特异性临床特征,诊断主要依靠病理学检查及血清IgG4检查,主要治疗方法为手术以及使用糖皮质激素,需要长期随访。

关键词: IgG4, Mikulicz病, 自身免疫性疾病, 颌下腺肿大, 组织纤维化, 糖皮质激素