Akkelle Bilge Şahin, Tutar Engin, Ergelen Rabia, Çelikel Çiğdem A, Ertem Deniz
Department of Pediatric Gastroenterology, Hepatology and Nutrition, Marmara University Faculty of Medicine, İstanbul, Turkey.
Department of Radiology, Marmara University Faculty of Medicine, İstanbul, Turkey.
Turk Pediatri Ars. 2020 Jun 19;55(2):191-194. doi: 10.14744/TurkPediatriArs.2019.83435. eCollection 2020.
Autoimmune pancreatitis has been described as a pancreatic manifestation of immunoglobulin G4-related disease, which is characterized by typical histopathologic, radiologic, and clinical features. Immunoglobulin G4-related disease is usually accompanied by elevated serum immunoglobulin G4 level, and can involve multiple organ/systems. Immunoglobulin G4-related disease has rarely been reported in pediatric population. There are few reports of inflammatory bowel disease in association with immunoglobulin G4-related disease. We describe a 7-year-old girl who presented with pancreatitis and concurrent sclerosing cholangitis, and developed bloody diarrhea during follow-up. An endoscopic examination revealed inflammatory bowel disease, and later lacrimal gland involvement was also recognized. She was diagnosed as having immunoglobulin G4-related disease, and her clinical signs and symptoms improved dramatically after steroid treatment. Hence, awareness of the clinical picture is important and early diagnosis can prevent fibrosis and organ damage.
自身免疫性胰腺炎已被描述为免疫球蛋白G4相关疾病的胰腺表现，其特征为典型的组织病理学、放射学和临床特征。免疫球蛋白G4相关疾病通常伴有血清免疫球蛋白G4水平升高，可累及多个器官/系统。免疫球蛋白G4相关疾病在儿科人群中鲜有报道。关于炎症性肠病与免疫球蛋白G4相关疾病相关的报道也很少。我们描述了一名7岁女孩，她出现胰腺炎并并发硬化性胆管炎，随访期间出现血性腹泻。内镜检查显示为炎症性肠病，后来还发现泪腺受累。她被诊断为患有免疫球蛋白G4相关疾病，类固醇治疗后其临床体征和症状显著改善。因此，了解临床表现很重要，早期诊断可预防纤维化和器官损害。
