中文摘要：

目的通过分析1例累及气管及气管周围软组织IgG4相关性疾病的临床资料并复习文献，提高对该病的认识与诊治水平。方法分析1例累及气管及气管周围软组织的IgG4相关性疾病患者的临床表现、实验室检查及影像、组织病理、治疗及预后，并进行相关文献复习。结果患者女，18岁，因咳嗽、呼吸困难2月余，发现颈部肿物1月余入院。颈部CT显示，颈段气管不规则狭窄，气管周围软组织广泛弥漫增厚。气管旁肿物活检病理显示，增生的致密胶原纤维组织，见较多淋巴细胞、浆细胞浸润，免疫组化：IgG4阳性浆细胞数〉50个/HP，IgG4阳性细胞数/IgG阳性细胞数〉40%。血清IgG4水平2 930 mg/L，诊断IgG4相关性疾病。静脉点滴甲泼尼龙80 mg/d，3 d后序贯口服泼尼松40 mg/d，症状缓解，1个月后复查颈部CT显示，颈段气管狭窄明显改善，气管周围软组织明显缩小。检索英文文献，共20例累及气管及气管周围软组织的IgG4相关性疾病，仅单纯累及气管壁及气管旁软组织者1例，同时伴有气管外受累者19例。12例检测血清IgG4，11例升高（6 150～34 050 mg/L）。18例患者接受口服糖皮质激素治疗，部分联合免疫抑制剂或利妥昔单抗治疗，预后良好。结论累及气管及气管周围软组织的IgG4相关性疾病临床少见，应积极完善组织病理学检查，糖皮质激素治疗有效，预后相对良好。

英文摘要：

Objective To investigate the clinical data of a patient with IgG4-related disease involving the trachea and paratracheal soft tissue and review the literature so as to improve the understanding level of the disorder.Methods To analyze the clinical manifestation, laboratory examination, imaging, histopathology, treatment and prognosis of a patient with IgG4-related disease trachea and paratracheal soft tissue involved, who was admitted to the Department of Respiratory and Critical Care Medicine at Beijing Chaoyang Hospital. The relevant literatures were reviewed.Results A 18-year-old female was admitted with chief complaint of cough, dyspnea, and neck mass. Neck CT suggested that tracheal stenosis was caused by surrounded soft tissue. Paratracheal mass biopsy showed dense collagen fibers with infiltration of many lymphocytes and plasma cells. Immunohistochemical stain found that IgG4-positive plasma cells were 〉50/high power field （HPF） and a ratio of IgG4/IgG positive cells was over 40% .The level of serum IgG4 was significantly increased （2 930 mg/L）. She was diagnosed as IgG4-related disease. The patient was treated with 80 mg intravenous methylprednisolone per day for three days, then prednisone 40 mg daily oral. Her dyspnea was significantly relieved.One month later, CT scan showed that the cervical tracheal stenosis was significantly improved. We identified 20 cases of IgG4-related disease involving the trachea and paratracheal soft tissue from databases, in which only 1 case was similar as this patient. The other 19 cases were of extratracheal involvement. Elevated serum IgG4 was detected in 11/12 patients. Most patients were treated with glucocorticoid, some combined with immunosuppressive agents and rituximab. The clinical outcome was good.Conclusion IgG4-related disease involving the trachea and paratracheal soft tissue is a rare condition. Serum IgG4 level and histopathology should be considered for diagnosis. Glucocorticoid is effective.