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Abstract: IgG4 related disease(IgG4-RD)is a newly recognized chronic fibroinflammatory disease in recent years.It is often accompanied by the significant elevation of serum IgG4 level, but the diagnostic specificity of the elevation is not high.The diagnosis is mainly based on histopathology, which is characterized by dense IgG4 positive plasma cell infiltration, storiform fibrosis and obliteran phlebitis.IgG4-RDcan involve various organs of the body, but less involve the nasal cavity and paranasal sinuses.The nasal symptoms of IgG4-RD are lack of specificity, so it is easier to be missed and misdiagnosed.PET/CT has important value in the diagnosis and differential diagnosis of the disease.We reviewed the relevant studies of IgG4-RD in nasal cavity and paranasal sinus in recent years, in order to improve the understanding of IgG4-RD in nasal cavity and paranasal sinuses and to improve the diagnosis and treatment rate.
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Key words:
- IgG4 related diseases /
- nasal cavity /
- paranasal sinuses
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表 1 累及鼻腔鼻窦IgG4-RD患者临床资料
作者 时间/年 年龄/岁 性别 临床表现 累及部位 血清IgG4/ (mg·dL-1) 组织病理学 治疗 随访 Rodriguez Fonseca等[7] 2020 52 女 鼻出血、面部不适 左上颌窦、鼻中隔、硬腭、牙槽突 336 淋巴浆细胞浸润，IgG4阳性浆细胞>10个，并伴有席纹状纤维化 类固醇+甲氨蝶呤+利妥昔单抗8个月 随访1年后复发 陈艺文等[8] 2020 63 男 间歇性鼻塞，伴少量流涕、打喷嚏、左眼视力改变 双侧上颌窦、筛窦，左侧额窦、蝶鞍、垂体 257 IgG4阳性浆细胞较多，IgG4+/IgG+细胞约40%，IgG4>10个/HPF 手术+泼尼松片30 mg/d并定期减量 随访1年未复发 Ji等[9] 2020 55 男 鼻塞、呼吸困难 鼻中隔、气管 332 大量淋巴细胞和浆细胞浸润，IgG4阳性的浆细胞>40个/HPF，IgG4+/IgG+比值>40% 类固醇 随访6个月未复发 Bashyam等[10] 2018 74 女 右侧脸颊肿胀，眼球突出 右上颌窦，鼻腔 160 丰富的浆细胞浸润，IgG+浆细胞明显增多，免疫组织化学表达IgG4(70%) 低剂量泼尼松龙+麦考酚酯 随访2年未复发 廖波等[11] 2017 53 男 双侧鼻塞、鼻出血伴右眼疼痛 全组鼻窦、鼻腔、鼻咽和鼻中隔 311 间质内淋巴细胞、中性粒细胞、浆细胞浸润，IgG4+及IgG+细胞数85/118高倍镜视野，IgG4+/IgG+比例72% 甲泼尼龙80 mg逐渐减量+糖皮质激素喷鼻 随访3个月未复发 Chen[12] 2016 36 男 右侧鼻部、前额顽固性疼痛 右前筛窦和中鼻甲 162 致密的淋巴浆细胞浸润，席纹状纤维化，闭塞性静脉炎以及IgG4+浆细胞>100个细胞 手术+泼尼松龙的起始剂量0.6 mg/kg，4周后逐渐减量 未报道 时文杰等[13] 2016 75 男 左眼肿痛、视力下降，左侧鼻腔脓涕、喷嚏 左侧筛窦 47.9 间质内淋巴细胞浸润，IgG4阳性细胞数>62 手术+甲泼尼龙+环磷酰胺 随访1年未复发 Vandjelovic等[14] 2016 64 男 右侧鼻出血，面部疼痛，鼻塞，流涕和鼻痂 右侧筛窦 未检测 致密浆细胞浸润，伴有纤维化，IgG4+/IgG>50% 手术+鼻腔冲洗+泼尼松30 mg/d逐渐减量 随访1年未复发 Inoue等[15] 2016 70 男 反复左鼻出血 左侧全鼻窦和中鼻甲 198 浆细胞密集浸润和席纹状纤维化，大多数浆细胞IgG阳性，IgG4/IgG阳性细胞59%，IgG4阳性细胞HPF>34个细胞 泼尼松0.6 mg，4周后逐渐减量 随访1年病情稳定 Suliman等[16] 2015 38 女 头痛、视力模糊 蝶窦 530 淋巴浆细胞浸润并伴有纤维化。IgG4>100/HPF，IgG4/IgG>40% 手术+类固醇 随访9个月未复发 Morri等[17] 2015 34 男 双侧鼻塞和右外鼻肿胀 鼻中隔 230 浆细胞浸润，伴有闭塞性静脉炎，IgG4>50/HPF，IgG4+/IgG>40% 泼尼松40 mg+甲氨蝶呤、叶酸 随访18个月未复发 Song等[18] 2015 72 男 左眼球突出症，眼眶周围疼痛，泪溢，鼻涕倒流 左上颌窦、筛窦 94.5 致密的淋巴浆细胞浸润和席纹状纤维化，IgG4>50/HPF 手术+泼尼松每天40 mg逐渐减量 随访6个月未复发 Prabhu等[19] 2014 15 女 鼻出血、鼻塞 鼻中隔，右侧鼻腔外侧壁，右侧上颌窦 206 致密淋巴细胞浆细胞浸润伴纤维化，IgG4+/IgG>50% 泼尼松龙 未报道 Prabhu等[19] 2014 15 女 右侧涕中带血、面部肿胀、张口受限 右侧鼻壁，鼻腔顶部，鼻中隔，右上颌骨，筛窦，蝶窦和腹膜后 579 致密淋巴细胞浆细胞浸润伴纤维化，IgG4+/IgG>50%，IgG4阳性细胞>30/HPF 泼尼松龙+利妥昔单抗 未报道 Cain等[20] 2014 62 女 鼻出血 鼻中隔、筛窦 未检测 IgG4浆细胞50/HPF 泼尼松20 mg 未报道 Cain等[20] 2014 79 男 鼻出血 鼻中隔前下部与上颌骨之间 未检测 浆细胞浸润伴纤维化，IgG4浆细胞30~50/HPF 布地奈德喷鼻 未报道 Chandrasekharan等[21] 2013 31 男 反复额部疼痛、恶心、发热，失明 蝶窦 未检测 纤维化伴浆细胞浸润，IgG4阳性的浆细胞为20~30/HPF，IgG4+/IgG浆细胞为35%~40%。 手术+甲泼尼龙+环磷酰胺 随访2个月未复发 Hu等[22] 2013 63 男 鼻塞、反复鼻窦感染 左侧上颌窦、筛窦、额窦 88 淋巴浆细胞浸润伴纤维化，IgG4染色阳性，Kappa阳性浆细胞为主 手术+糖皮质激素+利妥昔单抗 随访8个月未复发 Suzuki等[23] 2013 58 男 鼻塞、下颌肿胀 双侧上颌窦、筛窦、颌下腺、胰腺、胆管、腹膜后 2970 IgG4+/IgG约60.5，IgG4阳性细胞>234/HPF 泼尼松30 mg/d 未报道 Lindau等[24] 2013 69 男 慢性鼻窦炎、复视 右侧上颌窦 75 纤维化伴IgG4阳性浆细胞浸润，IgG4>30/HP 手术+利妥昔单抗+地塞米松，泼尼松无反应 未报道 Alt等[25] 2012 38 女 持续性额头痛 蝶窦 正常 纤维化的急性和慢性炎症，IgG4+细胞>150/HPF 手术+氟替卡松 术后2个月复发 Sasaki等[26] 2011 74 男 鼻塞、右脸颊肿胀 双侧上颌窦、鼻腔 114 浆细胞浸润；浆细胞表达IgG4 泼尼松龙40 mg逐渐减量 病情稳定 Ikeda等[27] 2010 50 女 血涕和鼻涕倒流 左上颌窦、筛窦、胸膜 258 弥漫纤维化伴淋巴细胞和浆细胞浸润；大量IgG4阳性浆细胞 泼尼松龙30 mg逐渐减量 随访6个月未复发 Pace等[28] 2010 73 男 右侧面部肿胀、低热 右侧上颌窦 正常 纤维化的慢性炎症，IgG4+/IgG>50% 泼尼松龙20 mg 随访5个月未复发 Ishida等[5] 2009 73 男 鼻塞 右上颌窦、鼻中隔、腮腺 63.4 纤维化伴淋巴浆细胞浸润和闭塞性静脉炎；大量的IgG4阳性浆细胞浸润，IgG4/IgG阳性细胞>70% 手术 随访11个月未复发 
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| 引用本文: |
卜春艳, 薛金梅, 赵长青, 等. 累及鼻腔鼻窦IgG4相关性疾病的研究进展[J]. 临床耳鼻咽喉头颈外科杂志, 2021, 35(11): 1042-1047. doi: 10.13201/j.issn.2096-7993.2021.11.018
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| Citation: |
BU Chunyan, XUE Jinmei, ZHAO Changqing, et al. Progress of IgG4 related sino-nasal diseases[J]. J Clin Otorhinolaryngol Head Neck Surg, 2021, 35(11): 1042-1047. doi: 10.13201/j.issn.2096-7993.2021.11.018
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