Diffuse Lung Disease: Connective Tissue-Associated Interstitial Lung Disease |

Frequent Cough in Scleroderma-Related Interstitial Lung Disesae (SSc-ILD): Characterisitcs and Response to Potentially Disease-Modifying Therapy in a Randomized Controlled Trial (RCT) (Scleroderma Lung Study II) FREE TO VIEW

Donald Tashkin, MD; Elizabeth Volkmann, MD; Dinesh Khanna; Michael Roth; Arthur Theodore; Bingling Wang; Chi-Hong Tseng; Robert Elashoff
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University of California Los Angeles, Los Angeles, CA

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

Chest. 2016;150(4_S):473A. doi:10.1016/j.chest.2016.08.487
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SESSION TITLE: Connective Tissue-Associated Interstitial Lung Disease

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 26, 2016 at 01:30 PM - 02:30 PM

PURPOSE: Using data from a 2-year RCT, we determined the proportion of SSc-ILD patients with frequent cough, its impact on cough-specific quality of life, correlation with other features of SSc-ILD, response to treatment for SSc-ILD (myclophenolate [MMF] or cyclophosphamide [CYC]) and the correlation between persistence or resolution of frequent cough and changes in physiologic and radiographic measures of ILD severity.

METHODS: 142 patients with SSc-ILD randomized into the Scleroderma Lung Study II (SLS II), a double-blind trial of MMF vs. oral CYC in symptomatic patients with progressive SSc-ILD (69 MMF; 73 CYC) underwent assessments of chronic cough (St. George’s Respiratory Questionnaire and Leicester Cough Questionnaire [LCQ], a cough-specific quality of life instrument), gastro-esophageal reflux disease (GERD) (UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract questionnaire [UCLA SCTC GIT], breathlessness (Visual Analog Scale [VAS]), other quality of life measures (SF-36), lung function (FVC, DLCO), and extent of lung fibrosis and ILD on thoracic HRCT (QLF and QILD) by quantittive image analysis. All assesssments were repeated every 3 months, except for the HRCT, performed at baseline and 24 months.

RESULTS: Of the 142 randomized subjects, 87 (61.3%) had frequent cough (on several or most days a week for the past 3 months). Of the 85 patients with frequent cough with data on GERD, 56 had GERD at baseline (66%), while GERD was present in only 19 of the 54 patients without frequent cough (35%) (p=0.00077; Chi-square). Average severity of GERD on a scale of 0-3.00 (UCLA GIT) was severe in both those with (score 1.69 ± 0.59) and without (score 1.38 ± 0.38) frequent cough, but significantly more severe in those with versus those without frequent cough (p=0.000). Compared with those without cough, breathing difficulty (VAS) was significantly worse, DLCO was significantly lower and the quantitative extent on HRCT of fibrosis (QLF) and total ILD (QILD) was significantly greater in those with frequent cough. Age, weight, duration of SSc and general health status did not differ significantly between those with and without frequent cough. Cough-speciffic quality of life (LCQ) was.relatively mildly impaired.on average.Of the 66 patients with frequent cough at baseline for whom cough data were collected at 24 months, 28 no longer reported frequent cough at 24 months (42.4% reduction) irrespective of MMF or CYC treatment.. Logistic regression did not reveal any significant relationship between a reduction in cough over the 24 month trial and improvements in FVC%, breathlessness or HRCT measures of extent of fibrosis or total ILD.

CONCLUSIONS: Frequent cough is common in patients with SSc-ILD and appears to be associated with both the presence of GERD and the severity of the underlying ILD. Frequent cough in SSc-ILD ofren resolves over a 2-year period with immunosuppressive therapy for SSc-ILD in parallel with improvements in FVC% predicted and dyspnea.

CLINICAL IMPLICATIONS: While frequent cough in SSc-ILD is partly explained by the presence of GERD, the substantial decline with immunosuppressive therapy suggests that the course of frequent cough in SSc-ILD may be a useful surrogate marker for the overal response to therapy.

DISCLOSURE: The following authors have nothing to disclose: Donald Tashkin, Elizabeth Volkmann, Dinesh Khanna, Michael Roth, Arthur Theodore, Bingling Wang, Chi-Hong Tseng, Robert Elashoff

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