title: Effective Use of Calcineurin Inhibitor in Combination Therapy for Interstitial Lung Disease in Patients With Dermatomyositis and Polymyositis.
abstract: OBJECTIVE
  The current study demonstrated the potential use of calcineurin inhibitor (CNI) in combination therapy for interstitial lung disease (ILD) complicated with dermatomyositis (DM) and polymyositis (PM).
  
  
  METHODS
  Thirty DM/PM patients with ILD were enrolled in this study. Continuous intravenous administration of cyclosporine A (IV-CsA) was simultaneously started with corticosteroid in patients presenting more than two respiratory distress factors as follows: <70 mmHg of PaO2, percentage of vital capacity <70%, and/or exertional dyspnea. Other patients took CNI orally with corticosteroid. When a patient presented exacerbation of ILD, intravenous cyclophosphamide pulse therapy (IVCY) was additionally administrated. Clinical outcomes were compared with those of 21 patients who had been previously treated in our hospital before starting this study, as the historical comparison group.
  
  
  RESULTS
  Seven patients underwent IV-CsA, maintaining the drug concentration in blood between 300 and 350 ng/mL, and six of them had favorable outcomes without any adverse events. Eight out of the 30 patients additionally required IVCY. Meanwhile, the frequency of death ascribable to respiratory failure was 6.7%, which was significantly lower than that of the historical comparison group (P = 0.043). Furthermore, longer survival free from exacerbation and severe adverse event was significantly shown (P = 0.029).
  
  
  CONCLUSIONS
  For repressing the activity of ILD in DM/PM patients, stabilizing the blood concentration of CNI immediately is a reasonable treatment as well as initiating immunosuppressive therapy in the early phase of the illness. IV-CsA may be a useful option for achieving this purpose in patients with severe ILD.
authors.affiliation: From the Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Shimojima
authors.forename: Wataru
authors.initials: W
authors.lastname: Ishii
authors.forename: Masayuki
authors.initials: M
authors.lastname: Matsuda
authors.forename: Dai
authors.initials: D
authors.lastname: Kishida
authors.forename: Shu-Ichi
authors.initials: SI
authors.lastname: Ikeda
mesh_terms: D000328:Adult; D000368:Aged; D065095:Calcineurin Inhibitors / Q000008:administration & dosage / Q000097:blood; D002985:Clinical Protocols; D016572:Cyclosporine / Q000008:administration & dosage*; D003882:Dermatomyositis / Q000150:complications*; D016903:Drug Monitoring / Q000379:methods; D004359:Drug Therapy, Combination / Q000379:methods; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007564:Japan / Q000453:epidemiology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology; D008297:Male; D008875:Middle Aged; D062072:Patient Acuity; D017285:Polymyositis / Q000150:complications*; D012129:Respiratory Function Tests / Q000379:methods; D016896:Treatment Outcome
chemical_list: D065095:Calcineurin Inhibitors; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine