探讨IgG4相关性疾病（IgG4-RD）的临床特点、血清学、影像学、组织病理学及治疗、预后，为IgG4-RD的诊治提供依据。

回顾性分析2013年至2019年在山西医科大学第二医院诊治的76例诊断为IgG4相关性疾病患者的临床资料。

76例患者中男性39例（51.3%），女性37例（48.7%），平均年龄（56.1±11.8）岁。最常见的受累器官为颌下腺25例（32.9%），其他器官受累依次为唇腺12例（15.8%)、胰腺10例（13.2%）、腮腺9例（11.8%）、腹膜后纤维化6例（7.9%）、淋巴结4例（5.3%）、鼻窦2例（2.6%）、肺脏2例（2.6%）、肾脏2例（2.6%），胆道系统2例（2.6%）,皮肤1例（1.3%）、结肠1例（1.3%）。白介素6与IgG4水平呈正相关（r=0.483，P<0.01），干扰素γ与IgG4水平呈正相关（r=0.228，P=0.048）；白介素2、白介素4、白介素10、肿瘤坏死因子α（TNF-α）水平与IgG4无相关性（P均>0.05）。61例患者接受病理组织学检查，主要表现为淋巴细胞、浆细胞浸润及纤维化形成，IgG4阳性浆细胞每高倍镜视野下>10个，和（或）IgG4/IgG>40%。18例（23.7%）患者单用糖皮质激素治疗，22例（28.9%）患者激素联合免疫抑制剂治疗，34例（44.7%）患者激素联合免疫调节治疗，2例（2.6%）患者未治疗；69例（90.8%）患者初始治疗反应较好。

IgG4-RD最常受累器官是颌下腺、唇腺、胰腺及腮腺，应用糖皮质激素和免疫抑制剂治疗有效。联合检测白介素6、干扰素γ可能在IgG4-RD的致病过程中起一定的预测作用，并可能成为IgG4-RD临床治疗的新靶点。

To explore the clinical characteristics, serology, imaging, histopathology, treatment, and prognosis of IgG4-related diseases (IgG4-RD).

Clinical data of 76 patients diagnosed with IgG4-RD at the Second Hospital of Shanxi Medical University from 2013 to 2019 were retrospectively analyzed.

Among the 76 patients, there were 39 males (51.3%) and 37 females (48.7%), with an average age of (56.1±11.8) years old. The most common involved organ was the submandibular gland (32.9%, 25/76), followed by the labial gland (15.8%, 12/76), pancreas (13.2%, 10/76), parotid gland (11.8%, 9/76), retroperitoneum (7.9%, 6/76), lymph nodes (5.3%, 4/76), sinus (2.6%, 2/76), lung (2.6%, 2/76), kidney (2.6%, 2/76), bile duct (2.6%, 2/76), skin (1.3%, 1/76), and colon (1.3%, 1/76). IL-6 was positively correlated with IgG4 (r=0.483, P<0.01), Interferon-γ was positively correlated with IgG4 (r=0.228, P<0.05), IL-2, IL-4, IL-10 and TNF-α had no correlation with IgG4 (P>0.05). Histopathological examination was performed in 61 cases, which were mainly characterized by infiltration of lymphocytes and plasmacytes and fibrosis,>10 IgG4(+) plasma cells per high-power field, or ratio of IgG4(+)/IgG(+) cells > 40%. Of all patients included, 23.7%(18/76) were treated with glucocorticoid alone, 28.9% (22/76) were treated with hormone combined with immunosuppressive therapy, 44.7% (34/76) were treated with hormone combined with immunoregulatory therapy, 2.6% (2/76) were untreated, and 90.8% (69/76) responded well to initial treatment.

IgG4-RD is an autoimmune disease with multiple organ involvement. The most frequently involved organs are the submandibular gland, labrum gland, pancreas, and parotid gland. Combined detection of IL-6 and interferon-γ may play a predictive role in the pathogenesis of IgG4-RD, and they may become new targets for clinical treatment of IgG4-RD.