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