Abstract
IgG4-related disease was first defined in autoimmune pancreatitis but is known to affect other organs, including the biliary tree and liver. The aim of this study was to assess IgG4-positive plasma cells in a spectrum of chronic liver diseases.
Highlights
IgG4-related disease was first defined in autoimmune pancreatitis but is known to affect other organs, including the biliary tree and liver
Needle core biopsies were available for 3 Immunoglobulin G4-related disease (IgG4RD), 19 primary biliary cirrhosis (PBC), 22 autoimmune hepatitis (AIH), 18 primary sclerosing cholangitis (PSC), 25 HCV, and 1 secondary sclerosing cholangitis of unknown etiology cases (Table 1)
A significantly elevated (> 10) number of IgG4+ plasma cells per high power field (HPF) was only seen in the 3 IgG4RD cases
Summary
IgG4-related disease was first defined in autoimmune pancreatitis but is known to affect other organs, including the biliary tree and liver. The aim of this study was to assess IgG4-positive plasma cells in a spectrum of chronic liver diseases. Immunoglobulin G4-related disease (IgG4RD) is a systemic immune-mediated disease characterized by elevated serum IgG4 concentration and tissue infiltration by IgG4positive plasma cells [1,2,3,4]. Within the biliary tree and liver, IgG4RD is a known cause of sclerosing cholangitis, biliary strictures, sclerosing cholecystitis, gallbladder nodules and thickening, and inflammatory pseudotumors of the liver [4,5,6]. Among patients with systemic IgG4RD, 19.5% have biliary tree or gallbladder involvement and 4% have liver involvement.
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