Correlation of Cough With Disease Activity and Treatment With Cyclophosphamide in Scleroderma Interstitial Lung Disease

Abstract

Nonproductive cough is a characteristic symptom of interstitial lung diseases (ILDs), including scleroderma (SSc). In the Scleroderma Lung Study (SLS), a double-blind, randomized, placebo (PLA)-controlled trial of oral cyclophosphamide (CYC) administered for 1 year followed by an additional year of follow-up without treatment, 1 73% of the 158 study participants complained of cough at the time of enrollment, making it the second-most-common nonRaynaud symptom, exceeded only by dyspnea. After 12 to 18 months of therapy initiation, patients who received CYC showed signifi cant improvement in lung function, skin scores, dyspnea, disability, and various aspects of health-related quality of life, compared with those who received PLA. The benefi cial effects on all parameters except dyspnea were lost at 24 months (1 year after discontinuation of CYC). 2 The pathogenesis of cough in SSc and other ILDs is poorly understood. Cough is mediated through

Interstitial lung disease in South Africans with systemic sclerosis

“…These findings are consistent with previously reported international studies where cough was found to be a predictor of ILD activity and severity (51) , and both dyspnoea and bibasal crackles were predictors of FVC decline in SSc ILD patients (52) . Unfortunately due to lack of data, particularly with respect to grading of ILD severity on HRCT, further associations were not amenable to analysis.…”

“…Of interest in resource-poor settings, where HRCT (and possibly PFTs) may not be readily available, is the correlation between severity of lung fibrosis with clinical parameters at presentation -namely skin scores, dyspnoea and cough. Theodore et al, (2012) (51) found that cough correlated with severity of fibrosis, settled with treatment with cyclophosphamide, and returned with worsening disease activity.…”

Interstitial Lung Disease in Scleroderma

“…Of note, patients with PAH may also have exertional dyspnea and fatigue and the clinician must keep both entities in mind when evaluating an SSc patient for dyspnea. Dry cough is often present, and may correlate with DLCO and dyspnea[24]. In the Scleroderma Lung Study, cough severity improved in patients treated with cyclophosphamide, but these improvements disappeared after two years of follow up[25, 26].…”

Outcome Measures for Clinical Trials in Interstitial Lung Diseases

“…In IPF, cough is associated with poor HRQoL and disease progression [35]. In CTD-ILD, the prevalence of cough has been reported to be as high as 73% [36] and is associated with a poor quality of life, and worse extent of lung fibrosis [36]. The mechanism of cough in ILD is poorly understood.…”