Clinical differences between primary sclerosing cholangitis and sclerosing cholangitis with autoimmune pancreatitis.

Immunoglobin G4-hepatopathy: Association of immunoglobin G4-bearing plasma cells in liver with autoimmune pancreatitis

“…17 IgG4-related sclerosing cholangitis is similar to PSC with regard to cholangiographic findings, but recent reports have shown that these are in fact different clinical entities. 20 PSC patients in our control group had low serum IgG4 concentrations and lacked infiltrates of IgG4-bearing plasma cells.…”

Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 II. Extrapancreatic lesions, differential diagnosis

“…IgG4-related sclerosing cholangitis has shown a preponderance among older males and is frequently complicated with obstructive jaundice [51][52][53]. In contrast, PSC has been more commonly found in young and middle-aged patients, sometimes complicated with inflammatory bowel diseases [12,[51][52][53]. Cholangiography of IgG4-related sclerosing cholangitis reveals lower bile duct stenosis and relatively long strictures from the hilar to the intrahepatic biliary systems, with simple distal dilations [51,52].…”

“…Cholangiography of PSC characteristically shows band-like strictures (short strictures of 1-2 mm), a beaded appearance, a pruned tree appearance, or diverticulum-like outpouching (Fig. 5) [51,52,54]. Ultrasonography of IgG4-related sclerosing cholangitis has revealed wall thickening of the intra-or extrahepatic bile ducts.…”

“…In contrast, PSC has been more commonly found in young and middle-aged patients, sometimes complicated with inflammatory bowel diseases [12,[51][52][53]. Cholangiography of IgG4-related sclerosing cholangitis reveals lower bile duct stenosis and relatively long strictures from the hilar to the intrahepatic biliary systems, with simple distal dilations [51,52]. Cholangiography of PSC characteristically shows band-like strictures (short strictures of 1-2 mm), a beaded appearance, a pruned tree appearance, or diverticulum-like outpouching (Fig.…”

Prevalence of IgG-4-associated cholangiopathy based on serum IgG-4 levels in patients with primary sclerosing cholangitis and its relationship with inflammatory bowel disease

“…We found that with the exception of ascites, which had a significantly higher frequency in elevated serum IgG-4 patients, no significant differences existed in terms of other variables between PSC patients with normal and high serum IgG-4 levels. (16). Both PSC and IAC are more common in men than women.…”