title: Survival benefit associated with early cyclosporine treatment for dermatomyositis-associated interstitial lung disease.
abstract: Interstitial lung disease (ILD) is the most common cause of death in dermatomyositis (DM). Cyclosporine A (CsA) has shown to be effective in DM-associated ILD (DM-ILD). This study aimed to define the optimal time of CsA administration. A total of 47 patients with DM-ILD, who were treated with CsA at Seoul National University Hospital between January 1998 and June 2013, were enrolled. ILD was diagnosed based on typical chest high-resolution computed tomography (HRCT) findings. Patients with early and delayed CsA treatment were compared in regard to the mortality and ILD progression on HRCT. The early (n = 16) and the delayed treatment group (n = 31) did not differ in regard to baseline clinical characteristics including HRCT scores and pulmonary function. Patients with clinically amyopathic DM (CADM) were more common in the early treatment group. The mortality rate was significantly lower in the early treatment group than in the delayed treatment group (p = 0.009). The survival benefit of early CsA treatment remained significant even after adjusting for age, degree of dyspnea, CADM status, and the year of CsA treatment (hazard ratio 0.057, 95 % confidence interval 0.007-0.472). CsA stabilized disease progression on HRCT in the early treatment group (p = 0.738). Delay in CsA treatment is associated with a worse survival in patients with DM-ILD. Early CsA treatment should be considered at DM-ILD diagnosis especially in patients at a higher risk of developing a rapidly progressive ILD.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.|Department of Molecular Medicine and Biopharmaceutical Sciences, Graduate School of Convergence Science and Technology, Medical Research Institute, Seoul National University, Seoul, Korea.
authors.forename: Dong Jin
authors.initials: DJ
authors.lastname: Go
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.|Department of Molecular Medicine and Biopharmaceutical Sciences, Graduate School of Convergence Science and Technology, Medical Research Institute, Seoul National University, Seoul, Korea.
authors.forename: Jin Kyun
authors.initials: JK
authors.lastname: Park
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
authors.forename: Eun Ha
authors.initials: EH
authors.lastname: Kang
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Hyun Mi
authors.initials: HM
authors.lastname: Kwon
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
authors.forename: Yun Jong
authors.initials: YJ
authors.lastname: Lee
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.|Department of Molecular Medicine and Biopharmaceutical Sciences, Graduate School of Convergence Science and Technology, Medical Research Institute, Seoul National University, Seoul, Korea.
authors.forename: Yeong Wook
authors.initials: YW
authors.lastname: Song
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea. leb7616@snu.ac.kr.
authors.forename: Eun Bong
authors.initials: EB
authors.lastname: Lee
mesh_terms: D000328:Adult; D016572:Cyclosporine / Q000627:therapeutic use*; D003882:Dermatomyositis / Q000150:complications* / Q000401:mortality; D018450:Disease Progression; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D017563:Lung Diseases, Interstitial / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D013997:Time Factors
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D011239:Prednisolone; D008727:Methotrexate
keywords: Amyopathic dermatomyositis; Cyclosporine; Dermatomyositis; Interstitial lung disease; Survival