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Autoimmune cholangiopathy associated with systemic lupus erythematosus

Authors

  • Samuel N. Heyman,

    Corresponding author
    1. 1 Department of Medicine, The Hebrew University Medical School, H6adassah University Hospital, Mt. Scopus, and2 Department of Nephrology, and 3Pathology, Hadassah Hospital, Ein Kerem, Jerusalem, Israel
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  • 1 Galia Spectre,

    1. 1 Department of Medicine, The Hebrew University Medical School, H6adassah University Hospital, Mt. Scopus, and2 Department of Nephrology, and 3Pathology, Hadassah Hospital, Ein Kerem, Jerusalem, Israel
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  • 1 Suhail Aamar,

    1. 1 Department of Medicine, The Hebrew University Medical School, H6adassah University Hospital, Mt. Scopus, and2 Department of Nephrology, and 3Pathology, Hadassah Hospital, Ein Kerem, Jerusalem, Israel
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  • 1 Dvora Rubinger,

    1. 1 Department of Medicine, The Hebrew University Medical School, H6adassah University Hospital, Mt. Scopus, and2 Department of Nephrology, and 3Pathology, Hadassah Hospital, Ein Kerem, Jerusalem, Israel
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  • 2 Orit Pappo,

    1. 1 Department of Medicine, The Hebrew University Medical School, H6adassah University Hospital, Mt. Scopus, and2 Department of Nephrology, and 3Pathology, Hadassah Hospital, Ein Kerem, Jerusalem, Israel
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  • and 3 Zvi Ackerman 1

    1. 1 Department of Medicine, The Hebrew University Medical School, H6adassah University Hospital, Mt. Scopus, and2 Department of Nephrology, and 3Pathology, Hadassah Hospital, Ein Kerem, Jerusalem, Israel
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Prof S Heyman, Department of Medicine, Hadassah University Hospital, Mt. Scopus, P.O.Box 24035, Jerusalem 91240, Israel. Fax: (972) 2–5823515.
e-mail: Heyman@cc.huji.ac.il

Abstract

Abstract:  We report a 39-year-old female who presented over 11 years with autoimmune cholangiopathy associated with kaleidoscopic manifestations of systemic lupus erythematosus (SLE), including, arthritis, skin changes, pleuritis, diffuse proliferative glomerulonephritis, lymphadenopathy, splenomegaly, hyperglobulinemia, and major depression. While antimitochondrial antibodies (AMA) were absent, antinuclear (ANA) and anti-DNA antibodies were detected in high titres associated with hypocomplementemia. The patient also had vitamin B12 deficiency and antiphospholipid antibodies. The patient required steroids and repeated courses of cyclophosphamide for the management of lupus nephritis, and ursodeoxycholic acid (ursolite) administration resulted in amelioration of cholestatic laboratory abnormalities. This unusual case report and review of literature illustrate that immune liver disease may be an important clinical manifestation of SLE, especially autoimmune cholangiopathy.

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