title: Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis.
abstract: Interstitial lung disease (ILD) associated with polymyositis/dermatomyositis (ILD-PM/DM), including amyopathic dermatomyositis (ADM), is recognized as an important condition because it frequently causes death, despite intensive therapy with high-dose corticosteroid and immunosuppressive agents, such as cyclosporine A and cyclophosphamide. Intravenous immunoglobulin therapy (IVIG) has shown efficacy for myopathy associated with PM/DM, but its usefulness for ILD-PM/DM is unclear. This study was designed to investigate the efficacy of IVIG for refractory ILD-PM/DM. A review was made of medical charts of five patients (2 men and 3 women) who were treated with IVIG for refractory ILD-PM/DM resistant to high-dose corticosteroid and cyclosporine A and/or cyclophosphamide. One patient had acute ILD-PM and four patients had acute ILD-ADM. Of the five patients, one patient with ILD-PM and one patient with ILD-ADM survived. No adverse reactions were seen due to IVIG treatment. There were no critical differences in the clinical parameters and clinical courses between survivors and nonsurvivors. IVIG treatment is safe and could be an effective salvage therapy for refractory ILD-PM/DM in certain cases, suggesting that further controlled trials are worthwhile.
authors.affiliation: Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan. yuzo.0716@nifty.com
authors.forename: Yuzo
authors.initials: Y
authors.lastname: Suzuki
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hayakawa
authors.forename: Seiichi
authors.initials: S
authors.lastname: Miwa
authors.forename: Masahiro
authors.initials: M
authors.lastname: Shirai
authors.forename: Masato
authors.initials: M
authors.lastname: Fujii
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Gemma
authors.forename: Takafumi
authors.initials: T
authors.lastname: Suda
authors.forename: Kingo
authors.initials: K
authors.lastname: Chida
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D001992:Bronchoalveolar Lavage Fluid / Q000276:immunology; D016572:Cyclosporine / Q000627:therapeutic use; D003882:Dermatomyositis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality; D004351:Drug Resistance; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D012189:Retrospective Studies; D016879:Salvage Therapy; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D008727:Methotrexate