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

Bihl, Florian; Emmenegger, Urban; Reichen, Jürg; Neftel, Klaus A; Zimmermann, Arthur; Cerny, Andreas (2006). Macrophage activating syndrome is associated with lobular hepatitis and severe bile duct injury with cholestasis. Journal of hepatology, 44(6), pp. 1208-12. Amsterdam: Elsevier 10.1016/j.khep.2006.03.003

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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 response and local tissue damage. This communication suggests that a macrophage, T- and NK-cell network is operational in the pathogenesis of the cholangiocyte, hepatocyte and sinus endothelial cell damage in MAS.

Item Type:	Journal Article (Original Article)
Division/Institute:	04 Faculty of Medicine > Service Sector > Institute of Clinical Pharmacology and Visceral Research [discontinued] 04 Faculty of Medicine > Service Sector > Institute of Pathology
UniBE Contributor:	Reichen, Jürg, Zimmermann, Arthur, Cerny, Andreas
ISSN:	0168-8278
ISBN:	16618513
Publisher:	Elsevier
Language:	English
Submitter:	Factscience Import
Date Deposited:	04 Oct 2013 14:45
Last Modified:	05 Dec 2022 14:14
Publisher DOI:	10.1016/j.khep.2006.03.003
PubMed ID:	16618513
Web of Science ID:	000237984400024
URI:	https://boris.unibe.ch/id/eprint/18557 (FactScience: 749)