摘要
IgG4相关性疾病(IgG4-related disease,IgG4-RD)是一种免疫介导的炎症伴纤维化疾病,其主要的组织病理学表现为IgG4阳性浆细胞浸润、闭塞性静脉炎和席纹状纤维化,且常伴有血清IgG4水平增高[1]。IgG4-RD的发现源于对自身免疫性胰腺炎(autoimmune pancreatitis,AIP)的研究。
To analyze the clinical features of IgG4-related diseases mainly with otologic manifestations and investigate diagnosis and treatment of IgG4-related diseases.We report the clinical course and diagnosis and treatment process of a case of IgG4-related disease misdiagnosed as chronic suppurative otitis media.After looking through relevant literatures,we review 22 cases of IgG4-related diseases mainly with otologic manifestations,and summarize their epidemiological characteristics,clinical manifestations,histopathologic features,radiologic features,and diagnostic and therapeutic criteria.IgG4-related diseases mainly with otologic manifestations are usually clinically characterized by atypical symptoms such as otalgia,tinnitus,aural fullness,otorrhea and progressive or fluctuating hearing loss,together with nausea,vomiting,and dizziness.The three central histopathologic features are dense lymphoplasmacytic infiltration,storiform fibrosis and obliterative phlebitis.The exact diagnosis of IgG4-related diseases requires a combination of clinical manifestations,serology,histopathology and radiologic results.Glucocorticoids are currently the first-line approach for IgG4-related diseases.Otologic manifestations involved in IgG4-related diseases are poorly specific,which is likely to result in more misdiagnosis and treatment delay.Current golden standard for diagnosing IgG4-related diseases is the identification of characteristic histopathology.In addition,immunohistochemistry can help clinicians diagnose IgG4-related diseases as early as possible.Otologists should strengthen their understanding of otologic manifestations of IgG4-related diseases.Early diagnosis and treatments are the key factors in prognosis.
出处
《临床耳鼻咽喉头颈外科杂志》
CAS
北大核心
2019年第9期814-819,共6页
Journal of Clinical Otorhinolaryngology Head And Neck Surgery
基金
国家重点研发计划资助项目“罕见病临床队列研究”(No:2016YFC0901500)
