1. IgG4 related disease in a seven year old girl with multiple organ involvement: A rare presentation
- Author
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Akkelle, Bilge Şahin, Tutar, Engin, Ergelen, Rabia, Çelikel, Çiğdem A., and Ertem, Deniz
- Subjects
child ,Case Report / Olgu Sunumu ,Autoimmune pancreatitis ,immunoglobulin G4-related disease - Abstract
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.
- Published
- 2020