Open AccessConcurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis
Author(s) -
Takehiro Kato,
Atsumasa Komori,
Sung-Kwan Bae,
Kiyoshi Migita,
Masahiro Ito,
Yasuhide Motoyoshi,
Seigo Abiru,
Hiromi Ishibashi
Publication year - 2012
Publication title -
world journal of gastroenterology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.427
H-Index - 155
eISSN - 2219-2840
pISSN - 1007-9327
DOI - 10.3748/wjg.v18.i2.192
Subject(s) - primary sclerosing cholangitis , amyloidosis , medicine , cholestasis , pathology , gastroenterology , primary biliary cirrhosis , serum amyloid a , systemic disease , disease , inflammation
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.