title: Macrophage activation syndrome in a patient with pulmonary inflammatory myofibroblastic tumour.
abstract: We describe for the first time a case of macrophage activation syndrome (MAS) in a patient with a history of inflammatory myofibroblastic tumour (inflammatory pseudotumour, IPT) of the lung and thoracic spine. The patient was admitted to the intensive care unit with a history of prolonged remitting fever, hepatosplenomegaly, bilaterally enlarged thoracic lymph nodes and an acute severe inflammatory response syndrome (SIRS). Up-regulated cytokine production (e.g. IL-1ß and IL-6), increased levels of ferritin and circulating soluble interleukin-2 receptor (sIL-2R, sCD25) led to the differential diagnosis of MAS. Bone marrow aspiration, the main tool for a definite diagnosis, revealed macrophages phagocytosing haematopoietic cells. Immunosuppressive therapy with corticosteroids and cyclosporine was an effective treatment in this patient.
authors.affiliation: Department of Nephrology and Clinical Immunology, University Hospital of the Aachen University of Technology (RWTH), Aachen, Germany. ckuppe@ukaachen.de.
authors.forename: Christoph
authors.initials: C
authors.lastname: Kuppe
authors.forename: Saskia
authors.initials: S
authors.lastname: Westphal
authors.forename: Eva
authors.initials: E
authors.lastname: Bücher
authors.forename: Marcus J
authors.initials: MJ
authors.lastname: Moeller
authors.forename: Bernhard
authors.initials: B
authors.lastname: Heintz
authors.forename: Marion E
authors.initials: ME
authors.lastname: Schneider
authors.forename: Jürgen
authors.initials: J
authors.lastname: Floege