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Acute severe hepatitis in adult-onset Still’s disease: case report and comprehensive review of a life-threatening manifestation

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Abstract

Acute severe hepatitis is a rare complication of adult-onset Still’s disease (AOSD). This condition is poorly characterized. We performed a review of the medical literature to describe clinical, biological, pathological, and treatment characteristics from AOSD patients with acute severe hepatitis. Their characteristics were compared with AOSD patients without severe hepatitis. Twenty-one cases were collected including a new case reported here. Patients with severe hepatitis were mostly young adults with a median age of 28 years (range: 20 to 55 years). Overall, patients with severe hepatitis had less arthritis, macular rash, sore throat, lymphadenopathy, or splenomegaly than patients without severe hepatitis. Cytopenia was more frequent in case of severe hepatitis. Most patients were treated with steroids, and the use of biotherapies has increased over the last decade. Despite treatment, 49% of patients required liver transplantation and 24% died.

Key Points

• Acute severe hepatitis in adult-onset Still’s disease (AOSD) is associated with liver transplantation and/or death in, respectively, 43% and 24% of cases.

• Severe hepatitis is the inaugural manifestation of AOSD in half of cases. Diagnosis is difficult when extra-hepatic clinical manifestations are lacking.

• The mechanism of hepatic necrosis in AOSD with severe hepatitis is unknown. Liver biopsy is not specific and should not delay treatment initiation.

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Authors and Affiliations

  1. Service de médecine interne, Aix Marseille Université, APHM, Hôpital La Timone, Marseille, France

    Romain Muller, Antoine Briantais, Benoit Faucher, Emmanuelle Bernit, Julie Seguier, Benoit Meunier, Jean-Robert Harlé, Mikael Ebbo & Nicolas Schleinitz

  2. Service d’hépato-gastro-entérologie, Aix Marseille Université, APHM, Hôpital La Timone, Marseille, France

    Patrick Borentain

  3. Réanimation polyvalente et des pathologies du foie, Aix Marseille Université, APHM, Hôpital La Timone, Marseille, France

    Cyril Nafati & Valery Blasco

  4. Service de chirurgie digestive, Aix Marseille Université, APHM, Hôpital La Timone, Marseille, France

    Emilie Gregoire

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  1. Romain Muller
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Muller, R., Briantais, A., Faucher, B. et al. Acute severe hepatitis in adult-onset Still’s disease: case report and comprehensive review of a life-threatening manifestation. Clin Rheumatol 40, 2467–2476 (2021). https://doi.org/10.1007/s10067-020-05383-y

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