title: Effects of liposteroid on the hemophagocytic syndrome in systemic lupus erythematosus.
abstract: Hemophagocytic syndrome (HPS) is a life-threatening disorder characterized by pancytopenia and activation of macrophages. Recently, corticosteroid incorporated in lipid microspheres (liposteroid) has been reported to be taken up by macrophages and to suppress their functions. Here we present a case of systemic lupus erythematosus complicated by HPS that was successfully treated with liposteroid in addition to an oral corticosteroid and intravenous high-dose cyclophosphamide therapy. The serum levels of tumor necrosis factor-alpha and ferritin that have been reported to be associated with activity of macrophages remarkably reduced after liposteroid administration. This case suggests that liposteroid is useful for the treatment of HPS.
authors.affiliation: Department of Hematology, Nephrology and Rheumatology, Kinki University School of Medicine, Osaka-Sayama, Osaka, Japan. funauchi@med.kindai.ac.jp
authors.forename: M
authors.initials: M
authors.lastname: Funauchi
authors.forename: M
authors.initials: M
authors.lastname: Ohno
authors.forename: T
authors.initials: T
authors.lastname: Yamagata
authors.forename: Y
authors.initials: Y
authors.lastname: Nozaki
authors.forename: K
authors.initials: K
authors.lastname: Kinoshita
authors.forename: A
authors.initials: A
authors.lastname: Kanamaru
mesh_terms: D000284:Administration, Oral; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage*; D000328:Adult; D001707:Biopsy, Needle; D001853:Bone Marrow / Q000473:pathology; D003520:Cyclophosphamide / Q000008:administration & dosage*; D016207:Cytokines / Q000032:analysis; D003907:Dexamethasone / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D015616:Histiocytosis, Non-Langerhans-Cell / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007150:Immunohistochemistry; D007378:Interleukins / Q000032:analysis; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D016207:Cytokines; D007378:Interleukins; D003907:Dexamethasone; D003520:Cyclophosphamide