A case of primary sclerosing cholangitis with unique clinical features for a 5-year follow up

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  • Nakagawa Mina
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University Center for Interprofessional Education, Tokyo Medical and Dental University
  • Asahina Yasuhiro
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University Department of Liver Disease Control, Tokyo Medical and Dental University
  • Murakawa Miyako
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University Department of Clinical Laboratory, Tokyo Medical and Dental University
  • Nagata Hiroko
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University
  • Kaneko Shun
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University
  • Otani Satoshi
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University
  • Kawai-Kitahata Fukiko
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University
  • Nitta Sayuri
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University
  • Nishimura-Sakurai Yuki
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University
  • Itsui Yasuhiro
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University Department of Medical Education Research and Development, Tokyo Medical and Dental University
  • Azuma Seishin
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University
  • Kakinuma Sei
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University Department of Liver Disease Control, Tokyo Medical and Dental University
  • Imada Atsuko
    Department of Pathology, Tokyo Medical and Dental University
  • Itoh Takashi
    Department of Pathology, Tokyo Medical and Dental University
  • Taniai Makiko
    Department of Internal Medicine and Gastroenterology, Tokyo Women's Medical University
  • Hashimoto Etsuko
    Department of Internal Medicine and Gastroenterology, Tokyo Women's Medical University
  • Tanaka Yujiro
    Department of Medical Education Research and Development, Tokyo Medical and Dental University
  • Watanabe Mamoru
    Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University

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Other Title
  • 5年にわたる経時的な経過観察の中で急激なエコー上変化をきたした原発性硬化性胆管炎の1例
  • 症例報告 5年にわたる経時的な経過観察の中で急激なエコー上変化をきたした原発性硬化性胆管炎の1例
  • ショウレイ ホウコク 5ネン ニ ワタル ケイジテキ ナ ケイカ カンサツ ノ ナカ デ キュウゲキ ナ エコー ジョウ ヘンカ オ キタシタ ゲンパツセイ コウカセイタンカンエン ノ 1レイ

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<p>A 26-year old young woman, who had been pointed out to have liver dysfunction in a medical check, admitted to our hospital in 2008. At first, she was slight obesity and suspected to be non-alcoholic fatty liver disease (NAFLD), because serology for hepatitis B virus, hepatitis C virus and various autoimmune markers were negative in a screening blood examination. However, the hepatic dysfunction had continued after exercise and dietary therapy, and even after administration of ursodeoxycholic acid and statin preparation. Liver biopsies performed in 2010 and 2012 did not lead to a definitive diagnosis, but localized high-echoic distinct lesions appeared in 2012 in her whole liver as diffuse lesions with a significant rise of the tumor marker in 2013. Therefore, we performed a liver biopsy to rule out hepato-biliary malignancies. The liver biopsy specimen did not show any malignant finding, but histological examination showed an extremely rapid progression of liver fibrosis.</p><p>Cholangiographic imaging study such as magnetic resonance (MR) cholangiopancreatography and endoscopic retrograde cholangiopancreatography revealed diffusely dilated intrahepatic ducts with multifocal narrowing with diffuse ductal wall thickening and enhancement and confirmed the diagnosis of primary sclerosing cholangitis (PSC). In this report, we describe an unique case of PSC with detailed histological features and imaging studies for a 5-year follow up.</p>

Journal

  • Kanzo

    Kanzo 57 (8), 382-390, 2016

    The Japan Society of Hepatology

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