Overlapping syndrome of autoimmune hepatitis and primary sclerosing cholangitis associated with pyoderma gangrenosum and ulcerative colitis.

We describe the case of a 18-year-old male patient who first presented with decompensated cirrhosis, fever and generalized lymphadenopathy. He had abnormal results for liver biochemical tests, with a hepatitic-like picture and high titre of antinuclear antibodies. According to the scoring system pro...

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Publicado en:European Journal of Gastroenterology & Hepatology Vol. 11; no. 12; pp. 1421 - 1425
Autores principales: Koskinas, John, Rapts, Irene, Manika, Zisoula, Hadziyannis, Stephanos, Koskinas, J, Raptis, I, Manika, Z, Hadziyannis, S
Formato: case study Journal Article
Publicado: Lippincott Williams & Wilkins Dec1999
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Dec1999
      vid: 11
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      pub: Lippincott Williams & Wilkins
      place: Baltimore, Maryland
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        10.1097/00042737-199912000-00014
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        atl: Overlapping syndrome of autoimmune hepatitis and primary sclerosing cholangitis associated with pyoderma gangrenosum and ulcerative colitis.
      aug:
        au:
          Koskinas, John
          Rapts, Irene
          Manika, Zisoula
          Hadziyannis, Stephanos
          Koskinas, J
          Raptis, I
          Manika, Z
          Hadziyannis, S
        affil: Academic Department of Medicine, Hippokration General Hospital, Athens, Greece
      sug:
        subj:
          Cholangitis, Sclerosing Complications
          Hepatitis, Autoimmune Complications
          Hepatitis, Autoimmune Therapy
          Liver Transplantation
          Pyoderma Gangrenosum Complications
          Colitis, Ulcerative Complications
          Male
          Pyoderma Gangrenosum Drug Therapy
          Cyclosporine Therapeutic Use
          Immunosuppressive Agents Therapeutic Use
          Adolescence
          Cholangitis, Sclerosing Drug Therapy
          Adolescent: 13-18 years
          Male
      ab: We describe the case of a 18-year-old male patient who first presented with decompensated cirrhosis, fever and generalized lymphadenopathy. He had abnormal results for liver biochemical tests, with a hepatitic-like picture and high titre of antinuclear antibodies. According to the scoring system proposed by the International Autoimmune Hepatitis Group he had 'definite' autoimmune hepatitis and responded well to immunosuppressive treatment. One year later he developed pyoderma gangrenosum which was successfully treated with cyclosporine. Two years later he experienced bloody diarrhoea as a first presentation of ulcerative colitis. At that time both the cholestatic biochemical picture and the cholangiographic appearances of the biliary tree were consistent with primary sclerosing cholangitis. Despite the addition of azathioprine and ursodeoxycholic acid to his treatment regime he developed recurrent episodes of cholangitis and intractable pruritus for which he underwent successful liver transplantation.
      pubtype: Academic Journal
      doctype:
        case study
        Journal Article
      ougenre: Article
    language: English
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