IgG4-Negative Autoimmune Pancreatitis with Sclerosing Cholangitis and Colitis: Possible Association with Primary Sclerosing Cholangitis?

• Published in: Internal Medicine Vol. 47; no. 10; pp. 943 - 948
• Main Authors: Saeki, Keita, Hozawa, Shigenari, Miyata, Naoteru, Nishizawa, Toshihiro, Soma, Hiromitsu, Iwao, Yasushi, Kameyama, Kaori, Hibi, Toshifumi
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.01.2008
• Subjects: Administration, Oral; Allium cepa; Antibodies, Antinuclear - blood; Autoimmune Diseases - complications; Autoimmune Diseases - drug therapy; autoimmune pancreatitis; Cholangiopancreatography, Endoscopic Retrograde; cholangitis; Cholangitis, Sclerosing - complications; Cholangitis, Sclerosing - pathology; colitis; Colitis - complications; Colitis - drug therapy; Colitis - pathology; Humans; Immunoglobulin G - blood; Male; Middle Aged; Pancreatitis - complications; Pancreatitis - drug therapy; primary sclerosing cholangitis; Steroids - administration & dosage
• ISSN: 0918-2918; 1349-7235; 1349-7235
• DOI: 10.2169/internalmedicine.47.0613

We report a case of autoimmune pancreatitis (AIP) with cholangiography and histopathology showing features characteristic of primary sclerosing cholangitis (PSC) and colitis. A 55-year-old previously-healthy man was diagnosed with anti-nuclear antibody (ANA)-positive AIP according to the finding of serum biochemistry, abdominal US (ultrasonography), CT (computed tomography) and ERCP (endoscopic retrograde cholangiopancreatography). However, bead-like strictures of intrahepatic bile ducts were also found and liver tissue showed onion skin-like periductal fibrosis but no anti-IgG4-positive cells. In addition, colon fiberscopy showed a pancolitis similar to ulcerative colitis indicating that, in this case, there may be an association with PSC. Here, we report a rare case of IgG4-negative AIP with sclerosing cholangitis and colitis with many clinical features that support an association with PSC.