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

查看全文 作　　者：Takehiro [1]Kato;Atsumasa [1,2]Komori;Sung-Kwan [1]Bae;Kiyoshi [1,2]Migita;Masahiro [1,2]Ito;Yasuhide [1]Motoyoshi;Seigo [1]Abiru;Hiromi [1,2]Ishibashi 高影响力作者 机构地区：[1]Clinical Research Center,National Hospital Organization Nagasaki Medical Center,Kubara 2-1001-1,Omura,Nagasaki 856-8562,Japan;[2]Department of Hepatology,Nagasaki University Graduate School of Biomedical Sciences,Kubara 2-1001-1,Omura,Nagasaki 856-8562,Japan高影响力机构 出　　处：《World Journal of Gastroenterology》索引2012年第18卷第2期,共5页高影响力期刊 基　　金：Supported by Grants-in-Aid for Clinical Research from National Hospital Organization,Grants-in-Aid for Scientific Research from the Ministry of Health,Labour and Welfare of Japan 摘　　要：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. 关 键 词：淀粉样变性 并发系统 肝硬化 原发性 胆管 淀粉样蛋白 肠道疾病 诊断价值
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