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

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Akkelle B. S. , Tutar E. , Ergelen R. , Çelikel Ç. , Ertem D.

TURK PEDIATRI ARSIVI-TURKISH ARCHIVES OF PEDIATRICS, cilt.55, ss.191-194, 2020 (ESCI İndekslerine Giren Dergi) identifier identifier identifier

  • Cilt numarası: 55
  • Basım Tarihi: 2020
  • Doi Numarası: 10.14744/turkpediatriars.2019.83435
  • Sayfa Sayıları: ss.191-194


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.