Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis

World J Gastroenterol. 2012 Jan 14;18(2):192-6. doi: 10.3748/wjg.v18.i2.192.

Abstract

Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis, except epidemic schistosomiasis. Among them, primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues, giving rise to systemic amyloidosis, due to a progressive and unresolved inflammatory process, and its possible association with inflammatory bowel diseases. Nevertheless, only one such case has been reported in the literature to date. We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis, without any evidence of other inflammatory disorders. As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4, the presence of IgG4(+) plasma cells was examined systemically, resulting in unexpected documentation of Congo-red-positive amyloid deposits, but not IgG4(+) plasma cells, in the liver, stomach and salivary glands. Elevated serum IgG4 is the hallmark of IgG4-related disease, including IgG4-associated cholangitis, but it has also been demonstrated in certain patients with PSC. Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.

Keywords: AA amyloidosis; IgG4-associated cholangitis; Primary sclerosing cholangitis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Amyloid / blood
  • Amyloidosis / pathology*
  • Amyloidosis / physiopathology
  • Asians
  • Cholangiopancreatography, Endoscopic Retrograde
  • Cholangitis / immunology*
  • Cholangitis / pathology
  • Cholangitis / physiopathology*
  • Cholangitis, Sclerosing / pathology
  • Cholangitis, Sclerosing / physiopathology*
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunoglobulin G / blood*
  • Immunoglobulin Light-chain Amyloidosis

Substances

  • Amyloid
  • Immunoglobulin G