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Original Research: Signs and Symptoms of Chest Disease |

Improved Cough and Cough-Specific Quality of Life in Patients Treated for Scleroderma-Related Interstitial Lung Disease: Results of Scleroderma Lung Study II

Donald P. Tashkin, MD; Elizabeth R. Volkmann, MD; Chi-Hong Tseng, PhD; Michael D. Roth, MD; Dinesh Khanna, MD; Daniel E. Furst, MD; Philip J. Clements, MD; Arthur Theodore, MD; Suzanne Kafaja, MD; Grace Hyun Kim, PhD; Jonathan Goldin, MD; Edgar Ariolla, PharmD; Robert M. Elashoff, PhD
Author and Funding Information

FUNDING/SUPPORT: The Scleroderma Lung Study II was funded by the National Heart, Lung, and Blood Institute [R01 HL089758 and R01 HL 089901].

aDepartment of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA

bDepartment of Radiologic Sciences, David Geffen School of Medicine at UCLA, Los Angeles, CA

cDepartment of Pharmaceutical Services, David Geffen School of Medicine at UCLA, Los Angeles, CA

dDepartment of Biomathematics, David Geffen School of Medicine at UCLA, Los Angeles, CA

eDepartment of Medicine, University of Michigan Medical School, Ann Arbor, MI

fDepartment of Medicine, Boston University School of Medicine, Boston, MA

CORRESPONDENCE TO: Donald P. Tashkin, MD, Department of Medicine, David Geffen School of Medicine at UCLA, 10833 Le Conte Ave, Los Angeles, CA 90095


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2017;151(4):813-820. doi:10.1016/j.chest.2016.11.052
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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.

Trial Registry  ClinicalTrials.gov; No.: NCT00883129; URL: www.clinicaltrials.gov.

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