title: Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis.
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.
authors.affiliation: Clinical Research Center, National Hospital Organization Nagasaki Medical Center, Kubara 2-1001-1, Omura, Nagasaki 856-8562, Japan.
authors.forename: Takehiro
authors.initials: T
authors.lastname: Kato
authors.forename: Atsumasa
authors.initials: A
authors.lastname: Komori
authors.forename: Sung-Kwan
authors.initials: SK
authors.lastname: Bae
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Migita
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ito
authors.forename: Yasuhide
authors.initials: Y
authors.lastname: Motoyoshi
authors.forename: Seigo
authors.initials: S
authors.lastname: Abiru
authors.forename: Hiromi
authors.initials: H
authors.lastname: Ishibashi
mesh_terms: D000368:Aged; D000682:Amyloid / Q000097:blood; D000686:Amyloidosis / Q000473:pathology* / Q000503:physiopathology; D044466:Asian People; D002760:Cholangiopancreatography, Endoscopic Retrograde; D002761:Cholangitis / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology*; D015209:Cholangitis, Sclerosing / Q000473:pathology / Q000503:physiopathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D000075363:Immunoglobulin Light-chain Amyloidosis
chemical_list: D000682:Amyloid; D007074:Immunoglobulin G