Acute liver transplantation in a 41-year-old male patient presenting symptoms of adult-onset Still's disease

Author:

Gelley Fanni12,Doros Attila1,Micsik Tamás3,Fazakas János1,Fehérvári Imre1,Zádori Gergely1,Müller Zsófia4,Gelley András5,Nemes Balázs1

Affiliation:

1. 1 Department of Transplantation and Surgery, Semmelweis University, Budapest, Hungary

2. 5 Department of Transplantation and Surgery, Semmelweis University, Baross u. 23–25, H-1082, Budapest, Hungary

3. 2 1st Department of Pathology and Experimental Cancer Research, Budapest, Hungary

4. 3 Department of Infectology, Saint George Hospital, Székesfehérvár, Hungary

5. 4 Polyclinic of Hospitaller Brothers of Saint John of God, Budapest, Hungary

Abstract

Abstract Adult-onset Still's disease is a rare systemic non-infectious inflammatory disease of unknown aetiology. It is characterized by high spiking fever, sore throat, arthralgia, transient maculopapular rash, hepatosplenomegaly, liver cytolysis, weight loss, leukocytosis, neutrophilia, lymphadenopathy, myopathia and polyserositis. Mild or moderate liver involvement is common but fulminate liver failure is a rare manifestation. We report a 41-year-old male with undiagnosed adult Still's disease who underwent liver transplantation due to acute fulminate liver failure. He died 6 months after the liver transplantation in a septic condition. To date, six patients with adult Still's disease-related liver failure have been reported who required liver transplantation. We emphasize that adult Still's disease should be considered in the differential diagnosis of fulminate liver failure, especially in young adults with fever of unknown aetiology or typical features in the history.

Publisher

Akademiai Kiado Zrt.

Subject

General Medicine

Reference22 articles.

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2. Adult-onset Still’s disease: a study of 14 cases;Singh S.;Clin Rheumatol,2008

3. Preliminary criteria for classification of adult’s Still’s disease;Yamaguchi M.;J Rheumatol,1992

4. Adult-onset Still’s disease. Clinical course and outcome;Cush J.J.;Arthritis Rheum,1987

5. Adult-onset Still’s disease with submassive hepatic necrosis;Takami A.;Int Med,1995

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