title: Improved Cough and Cough-Specific Quality of Life in Patients Treated for Scleroderma-Related Interstitial Lung Disease: Results of Scleroderma Lung Study II.
abstract: BACKGROUND
  Cough is a common symptom of scleroderma-related interstitial lung disease (SSc-ILD), but its relationship to other characteristics of SSc-ILD, impact on cough-specific quality of life (QoL), and response to therapy for SSc-ILD have not been well studied.
  
  
  METHODS
  We investigated frequent cough (FC) in patients with SSc-ILD (N = 142) enrolled in the Scleroderma Lung Study II, a randomized controlled trial comparing mycophenolate mofetil (MMF) and oral cyclophosphamide (CYC) as treatments for interstitial lung disease (ILD). We determined the impact of FC on QoL (Leicester Cough Questionnaire [LCQ]), evaluated the change in FC in response to treatment for SSc-ILD, and examined the relationship between gastroesophageal reflux disease (GERD) and cough during the trial.
  
  
  RESULTS
  Study participants who reported FC at baseline (61.3%) reported significantly more dyspnea, exhibited more extensive ILD on high-resolution CT, had a lower diffusing capacity for carbon monoxide, and reported more GERD symptoms than did those without FC. Cough-specific QoL was modestly impaired in patients with FC (total LCQ score, 15.4 ± 3.7; normal range, 3-21 [higher scores indicate worse QoL]). The proportion of patients with FC at baseline declined by 44% and 41% over 2 years in the CYC and MMF treatment arms, respectively, and this decline was significantly related to changes in GERD and ILD severity.
  
  
  CONCLUSIONS
  FC occurs commonly in SSc-ILD, correlates with both the presence and severity of GERD and ILD at baseline, and declines in parallel with improvements in both ILD and GERD over a 2-year course of therapy. Frequent cough might serve as a useful surrogate marker of treatment response in SSc-ILD trials.
  
  
  BACKGROUND
  ClinicalTrials.gov; No.: NCT00883129; URL: www.clinicaltrials.gov.
authors.affiliation: Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA. Electronic address: dtashkin@mednet.ucla.edu.
authors.forename: Donald P
authors.initials: DP
authors.lastname: Tashkin
authors.affiliation: Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Elizabeth R
authors.initials: ER
authors.lastname: Volkmann
authors.affiliation: Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Chi-Hong
authors.initials: CH
authors.lastname: Tseng
authors.affiliation: Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Michael D
authors.initials: MD
authors.lastname: Roth
authors.affiliation: Department of Medicine, University of Michigan Medical School, Ann Arbor, MI.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.affiliation: Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
authors.affiliation: Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Clements
authors.affiliation: Department of Medicine, Boston University School of Medicine, Boston, MA.
authors.forename: Arthur
authors.initials: A
authors.lastname: Theodore
authors.affiliation: Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Suzanne
authors.initials: S
authors.lastname: Kafaja
authors.affiliation: Department of Radiologic Sciences, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Grace Hyun
authors.initials: GH
authors.lastname: Kim
authors.affiliation: Department of Radiologic Sciences, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Goldin
authors.affiliation: Department of Pharmaceutical Services, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Edgar
authors.initials: E
authors.lastname: Ariolla
authors.affiliation: Department of Biomathematics, David Geffen School of Medicine at UCLA, Los Angeles, CA.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Elashoff
mesh_terms: D003371:Cough / Q000188:drug therapy* / Q000209:etiology; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D005260:Female; D005764:Gastroesophageal Reflux / Q000150:complications; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000188:drug therapy*; D008297:Male; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000627:therapeutic use*; D011788:Quality of Life; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications / Q000188:drug therapy*; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D004791:Enzyme Inhibitors; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D009173:Mycophenolic Acid
keywords: cough; health-related quality of life; immunosuppressive therapy; interstitial lung disease; scleroderma