IgG4 related disease in a 7-year-old girl with multiple organ involvement: A rare presentation.


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Ertem Şahinoğlu D., Tutar E., Ergelen R. , Çelikel Ç.

TURK PEDİATRİ ARŞİVİ, cilt.55, sa.1, 2020 (ESCI İndekslerine Giren Dergi)

  • Yayın Türü: Makale / Vaka Takdimi
  • Cilt numarası: 55 Konu: 1
  • Basım Tarihi: 2020
  • Dergi Adı: TURK PEDİATRİ ARŞİVİ

Özet

Autoimmune pancreatitis (AIP) has been described as a pancreatic manifestation of immunoglobulin G4-related disease (IgG4-RD), which is characterized by typical histopathological, radiological and clinical features. IgG4-RD is usually accompanied by elevated serum IgG4 levels and can involve multiple organ/systems. There are few reports of inflammatory bowel disease (IBD) in association with IgG4-RD. IgG4-RD has rarely been reported in pediatric population. We describe a 7 year-old girl, presented with pancreatitis and concurrent sclerosing cholangitis, developed bloody diarrhea during follow-up. Endoscopic examination revealed IBD, and later lacrimal gland involvement was also recognized. She had the diagnosis of IgG4-RD, and her clinical signs and symptoms improved dramatically after the steroid treatment. Hence awareness of the clinical picture is important and early diagnosis can prevent fibrosis and organ damage.  

Key Words: Autoimmune pancreatitis, IgG4 related disease, child

 

Autoimmune pancreatitis (AIP) has been described as a pancreatic manifestation of immunoglobulin G4-related disease (IgG4-RD), which is characterized by typical histopathological, radiological and clinical features. IgG4-RD is usually accompanied by elevated serum IgG4 levels and can involve multiple organ/systems. There are few reports of inflammatory bowel disease (IBD) in association with IgG4-RD. IgG4-RD has rarely been reported in pediatric population. We describe a 7 year-old girl, presented with pancreatitis and concurrent sclerosing cholangitis, developed bloody diarrhea during follow-up. Endoscopic examination revealed IBD, and later lacrimal gland involvement was also recognized. She had the diagnosis of IgG4-RD, and her clinical signs and symptoms improved dramatically after the steroid treatment. Hence awareness of the clinical picture is important and early diagnosis can prevent fibrosis and organ damage.  

Key Words: Autoimmune pancreatitis, IgG4 related disease, child