Objectives: To discuss the clinical and radiological features of IgG4-related rhinosinusitis. Methods: In this retrospective research, the clinical data of 16 patients diagnosed with IgG4 related rhinosinusitis, who were enrolled in Beijing Tongren Hospital from November 2013 to September 2017, were collected. The clinical features, laboratory findings, radiological findings, histological features, treatment and prognosis were all summarized. Results: There were 12 males and 4 females among 16 patients, and male-to-female ratio was 3︰1. The age was between 30 to 70 years old, with median age of 52 years old. The chief nasal complaints were nasal obstruction and hyposmia, complicated with proptosis and eyelid swelling in 11 patients (11/16). Serum IgG4 levels were elevated in all patients and the value was over 1.44 g/L, and one patient serum IgG4 level was up to 49.70 g/L. Computed tomography (CT) showed the mainly affected sinuses were bilateral ethmoid sinus and olfactory cleft. The classic feature of CT scans was thickening of the involved bilateral ethmoid sinus mucous membrane with ethmoid bone absorption, which was mainly at midline. Histological features were severe inflammation of the mucosal tissue with mass of neutrophils and plasma cell infiltration. All patients were treated by methylprednisolone combined with cyclophosphamide or methotrexate. Remission of symptoms was detected in all patients. Conclusion: The clinical features of IgG4-related rhinosinusitis are often accompanied by orbital tissue involvement, elevated IgG4 serum concentration, associated sinus imaging changes, and sensitive glucocorticoid and immunosuppressive therapy.
目的： 初步探讨IgG4相关性鼻窦炎的临床和影像学特征。 方法： 回顾性分析2013年11月至2017年9月于北京同仁医院诊治的16例IgG4相关性鼻窦炎患者的临床资料，结合血清学结果、影像学资料及病理学检查，总结IgG4相关性鼻窦炎的临床特点及治疗转归。 结果： 16例患者中男性12例，女性4例，男女比为3︰1，年龄30~70岁，中位年龄52岁。主要鼻部症状为鼻塞及嗅觉减退，其中11例（11/16）同时伴有突眼、眼睑肿胀等眼部症状。所有患者血清IgG4均升高（>1.44 g/L），其中最高可达49.70 g/L。鼻窦CT可见主要受累部位为双侧筛窦及嗅裂区，以中线部位发病为主，表现为窦内大量软组织密度影伴骨质吸收。病理学特征为：黏膜组织呈重度炎症，间质可见较多中性粒细胞及浆细胞浸润，纤维组织增生。所有患者确诊后采用甲泼尼龙联合环磷酰胺或甲氨蝶呤治疗，症状均可缓解。 结论： IgG4相关性鼻窦炎的临床特征为发病时常伴有眶内组织受累，血清IgG4浓度升高，可伴有鼻窦影像学改变，糖皮质激素和免疫抑制剂治疗有效。.
Keywords: Disease attributes; IgG4-related disease; Sinusitis.