Kato T, Komori A, Bae SK, Migita K, Ito M, Motoyoshi Y, Abiru S, Ishibashi H. Concurrent systemic AA amyloidosis can discriminate primary sclerosing cholangitis from IgG4-associated cholangitis. World J Gastroenterol 2012; 18(2): 192-196 [PMID: 22253527 DOI: 10.3748/wjg.v18.i2.192]
Corresponding Author of This Article
Atsumasa Komori, MD, PhD, Clinical Research Center, National Hospital Organization Nagasaki Medical Center, and Department of Hepatology, Nagasaki University Graduate School of Biomedical Sciences, Kubara 2-1001-1, Omura, Nagasaki 856-8562, Japan. komori@nmc.hosp.go.jp
Article-Type of This Article
Case Report
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Takehiro Kato, Atsumasa Komori, Sung-Kwan Bae, Kiyoshi Migita, Masahiro Ito, Yasuhide Motoyoshi, Seigo Abiru, Hiromi Ishibashi, Clinical Research Center, National Hospital Organization Nagasaki Medical Center, Kubara 2-1001-1, Omura, Nagasaki 856-8562, Japan
Atsumasa Komori, Kiyoshi Migita, Masahiro Ito, Hiromi Ishibashi, Department of Hepatology, Nagasaki University Graduate School of Biomedical Sciences, Kubara 2-1001-1, Omura, Nagasaki 856-8562, Japan
Author contributions: Kato T, Komori A and Bae SK collected the data, and wrote the paper; Kato T, Komori A and Bae SK interpreted clinical and laboratory data; Ito M performed pathological diagnosis; Migita K performed PCR and sequencing; Kato T, Bae SK, Komori A and Motoyoshi Y analyzed imaging data; and Kato T, Komori A, Bae SK, Abiru S and Ishibashi H contributed to the diagnostic decision.
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
Correspondence to: Atsumasa Komori, MD, PhD, Clinical Research Center, National Hospital Organization Nagasaki Medical Center, and Department of Hepatology, Nagasaki University Graduate School of Biomedical Sciences, Kubara 2-1001-1, Omura, Nagasaki 856-8562, Japan. komori@nmc.hosp.go.jp
Telephone: +81-957-523101 Fax: +81-957-536675
Received: May 30, 2011 Revised: August 11, 2011 Accepted: August 15, 2011 Published online: January 14, 2012
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.
