Jariwala Mehul Pravinchandra, Agarwal Manjari, Mulay Kaustubh, Sawhney Sujata
Department of Pediatric Rheumatology, Sir Ganga Ram Hospital, New Delhi, 110060, India,
Indian J Pediatr. 2014 Oct;81(10):1108-10. doi: 10.1007/s12098-014-1471-y. Epub 2014 May 23.
IgG4 related systemic disease (IgG4-RSD) has been recognised in the last few years. Orbital pseudotumor as a presentation of IgG4-RSD is one of the rare complaints encountered in pediatric population. It is an inflammatory condition of unknown etiology characterized by tumorous swelling of the organs, characteristic histopathologic changes and elevated IgG4: IgG plasma cells ratio. The disease is also characterized by involvement of varied organ systems. The authors describe a seven-year-old boy with orbital pseudotumor after two years of initial onset with waxing and waning course, steroid responsive lesion and biopsy suggestive of IgG4-RSD involving the extraocular soft tissue. Treatment with oral corticosteroids and Azathioprine produced a significant decline in the pseudotumor size. It is important for pediatricians to be aware of this condition as appropriate recognition and management is important to prevent long-term damage of the tissue involved. This is the first case of IgG4 related orbital pseudotumor reported from India.
IgG4相关性系统性疾病（IgG4-RSD）在过去几年中已得到认可。眼眶假瘤作为IgG4-RSD的一种表现，是儿科人群中罕见的病症之一。它是一种病因不明的炎症性疾病，其特征为器官的肿瘤样肿胀、特征性组织病理学改变以及IgG4:IgG浆细胞比例升高。该疾病还具有累及多个器官系统的特点。作者描述了一名7岁男孩，最初发病两年后出现眼眶假瘤，病情有反复，病变对类固醇有反应，活检提示IgG4-RSD累及眼外软组织。口服皮质类固醇和硫唑嘌呤治疗使假瘤大小显著缩小。儿科医生了解这种疾病很重要，因为正确的识别和管理对于预防受累组织的长期损害很重要。这是印度报道的首例IgG4相关性眼眶假瘤病例。
