title: [Hepatic manifestation of a macrophage activation syndrome (MAS)].
abstract: BackgroundElevated liver values are the most common pathological laboratory result in Germany. Frequent findings, especially in younger patients, are nutritive- or medicamentous- toxic reasons, viral or autoimmune hepatitis. A macrophage activation syndrome (MAS) may manifest like a viral infectious disease with fever, hepatosplenomegaly and pancytopenia and is associated with a high mortality. It is based on an enhanced activation of macrophages with increased cytokine release, leading to organ damage and multi-organ failure. In addition to genetic causes, MAS is commonly associated with infections and rheumatic diseases. We report the case of a 26-year-old female patient suffering from MAS as a rare cause of elevated liver enzymes.MethodsPatient characteristics, laboratory values, liver histology, bone marrow and radiological imaging were documented and analyzed.Case ReportAfter an ordinary upper airway infection with bronchitis, a rheumatic arthritis appeared and was treated with leflunomide und methotrexate. In the further course of the disease, the patient developed an acute hepatitis with fever, pancytopenia and massive hyperferritinemia. Immunohistochemistry of the liver biopsy revealed hemophagocytosis and activation of CD68-positive macrophages. In the radiological and histological diagnostics of the liver and bone marrow, an MAS was diagnosed as underlying disease of the acute hepatitis. Under therapy with prednisolone, the fever disappeared and transaminases and ferritin rapidly normalized.ConclusionAside from the frequent causes of elevated liver values in younger patients, such as nutritive toxic, drug induced liver injury, viral or autoimmune hepatitis, especially in case of massive hyperferritinemia, a MAS should be considered as a rare cause of acute liver disease.
authors.affiliation: I. Medizinische Klinik und Poliklinik, Gastroenterologie und Hepatologie, Universitätsmedizin der Johannes Gutenberg Universität Mainz, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Nagel
authors.affiliation: Rheumatologie und Klinische Immunologie, Universitätsmedizin Mainz, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Schwarting
authors.affiliation: Institut für Pathologie, Universitätsmedizin Mainz, Germany.
authors.forename: Beate K
authors.initials: BK
authors.lastname: Straub
authors.affiliation: I. Medizinische Klinik und Poliklinik, Gastroenterologie und Hepatologie, Universitätsmedizin der Johannes Gutenberg Universität Mainz, Germany.
authors.forename: Peter R
authors.initials: PR
authors.lastname: Galle
authors.affiliation: I. Medizinische Klinik und Poliklinik, Gastroenterologie und Hepatologie, Universitätsmedizin der Johannes Gutenberg Universität Mainz, Germany.
authors.forename: Tim
authors.initials: T
authors.lastname: Zimmermann
mesh_terms: D000208:Acute Disease; D000328:Adult; D005260:Female; D005858:Germany; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis; D006801:Humans; D019189:Iron Metabolism Disorders / Q000150:complications*; D055501:Macrophage Activation Syndrome / Q000150:complications