Macrophage activating syndrome is associated with lobular hepatitis and severe bile duct injury with cholestasis

Journal of Hepatology
Florian BihlAndreas Cerny

Abstract

Macrophage activating syndrome (MAS) is a rare hematological disorder associated with uncontrolled systemic T-cell activation. Persistent fever, fatigue and hepatosplenomegaly are frequent clinical manifestations, whereas hyperferritinemia, elevated serum lactate dehydrogenase levels and cytopenia are key criteria for the diagnosis of MAS. The nature of liver pathology in MAS has been partially elucidated but destructive biliary lesions have been rarely described. This report illustrates four cases of MAS developing marked cholestasis, leading to one case of biliary cirrhosis necessitating liver transplantation. Histologically, liver involvement was characterized in all cases by acute lobular hepatitis, marked hepatocyte apoptosis and small bile duct injury similar to the vanishing bile duct syndrome. Immuno-histological studies showed that the inflammatory changes and bile duct lesions were dominated by the presence of activated macrophages and T-cells, in particular CD8+ lymphocytes, and in part NK-cells. These findings suggest that in MAS, various T-cell triggers such as infection, autoimmune disease and malignancy might result in the release of cytokines, which in turn activate macrophages to trigger a systemic acute phase ...Continue Reading

References

Oct 1, 1991·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·R H WiesnerR A Krom
Jan 1, 1993·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·S G HubscherE Elias
May 18, 2000·American Journal of Hematology·U EmmeneggerK A Neftel
Nov 15, 2000·Emerging Infectious Diseases·D N Fisman

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Citations

Dec 24, 2009·Pediatric Rheumatology Online Journal·Scott CannaLeonard L Dragone
Oct 5, 2013·Best Practice & Research. Clinical Gastroenterology·Lori Edwards, Ian R Wanless
Nov 20, 2016·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Estella M AlonsoEdward Doo
Sep 11, 2010·Expert Review of Clinical Immunology·Shumpei Yokota, Tadamitsu Kishimoto
Oct 22, 2014·Expert Review of Gastroenterology & Hepatology·Balázs NemesAttila Doros

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