title: Successful treatment of fulminant pulmonary hemorrhage associated with systemic lupus erythematosus.
abstract: We report a patient with systemic lupus erythematosus (SLE) who developed fulminant pulmonary hemorrhage. This patient also showed liver dysfunction, bicytopenia and hyperferritinemia, with an increase in serum levels of interleukin (IL)-1 beta, IL-6 and tumor necrosis factor-alpha (TNF-alpha) at the onset of pulmonary symptoms, probably indicating an associated hemophagocytic syndrome. Despite an acute progressive course temporarily requiring mechanical ventilation the patient was successfully treated with continuous drip infusion of tacrolimus, plasmapheresis and intravenous high-dose immunoglobulin and corticosteroid. In this patient increased inflammatory cytokines ascribable to activation of macrophages and/or helper T cells were considered to play an important role in the pathogenesis of the pulmonary hemorrhage. Because this complication is frequently fatal in SLE, intensive therapy, including immunosuppressants and plasmapheresis, should be actively considered as early as possible after onset.
authors.affiliation: Third Department of Medicine, Shinshu University School of Medicine, 3-1-1 Asahi, 390-8621 Matsumoto, Japan.
authors.forename: Ken-ichi
authors.initials: K
authors.lastname: Hoshi
authors.forename: Masayuki
authors.initials: M
authors.lastname: Matsuda
authors.forename: Mariko
authors.initials: M
authors.lastname: Ishikawa
authors.forename: Shigeaki
authors.initials: S
authors.lastname: Mitsuhashi
authors.forename: Takahisa
authors.initials: T
authors.lastname: Gono
authors.forename: Takao
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D005260:Female; D006470:Hemorrhage / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008171:Lung Diseases / Q000276:immunology* / Q000628:therapy*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology / Q000628:therapy; D010956:Plasmapheresis; D016559:Tacrolimus / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D016559:Tacrolimus