IgG4 related disease in a seven year old girl with multiple organ involvement: A rare presentation.

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.

• Authors: Bilge Şahin Akkelle, Engin Tutar, Rabia Ergelen, Çiğdem A Çelikel, Deniz Ertem
• Journal: Turk pediatri arsivi (Turk Pediatri Ars) Vol. 55 Issue 2 Pg. 191-194 ( 2020) ISSN: 1306-0015 [Print] Turkey
• PMID: 32684765 (Publication Type: Case Reports)
• Copyright: Copyright: © 2020 Turkish Archives of Pediatrics.