Impact of oral cyclophosphamide on health‐related quality of life in patients with active scleroderma lung disease: Results from the scleroderma lung study

Abstract

Objective. To assess the impact of cyclophosphamide (CYC) on the health-related quality of life (HRQOL) of patients with scleroderma after 12 months of treatment.Methods. One hundred fifty-eight subjects participated in the Scleroderma Lung Study, with 79 each randomized to CYC and placebo arms. The study evaluated the results of 3 measures of health status: the Short Form 36 (SF-36), the Health Assessment Questionnaire (HAQ) disability index (DI), and Mahler's dyspnea index, and the results of 1 preference-based measure, the SF-6D. The differences in the HRQOL between the 2 groups at 12 months were calculated using a linear mixed model. Responsiveness was evaluated using the effect size. The proportion of subjects in each treatment group whose scores improved at least as much as or more than the minimum clinically important difference (MCID) in HRQOL measures was assessed.Results. After adjustment for baseline scores, differences in the HAQ DI, SF-36 role physical, general health, vitality, role emotional, mental health scales, and SF-36 mental component summary (MCS) score were statistically significant for CYC versus placebo (P < 0.05). Effect sizes were negligible (<0.20) for all of the

Reliability, validity, and minimally important differences of the SF-6D in systemic sclerosis

“…Also, there was no association between SF-6D scores and our clinical measures (FVC% predicted and skin score). These findings are similar to those from the Scleroderma Lung Study [27,41]. At baseline, the SF-6D was able to discriminate between mild, moderate, and severe PGA (P < 0.001 for all the comparisons), and between mild HAQ-DI disability vs. moderate and severe HAQ-DI disability (P < 0.01).…”

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

“…23,32,33 This would be especially important in the few patients in whom the blind had to be removed because of the intention-to-treat principle, but these comprised only two patients who were unblinded after receiving a year of PLA and then received CYC. The cough score is a subjective measurement, the resolution of which may not be sufficiently sensitive to detect clinically relevant changes in cough frequency or severity.…”

Management of interstitial lung disease in systemic sclerosis: Lessons from SLS and FAST

“…Specifically, the mean absolute difference in adjusted 12-month FVC percent was 2.53%, a statistically significant but clinically modest effect (Table 1). Clinically and statistically significant differences favoring CYC were also seen for transitional dyspnea index (TDI) (P < 0.001), disability index of the Health Assessment Questionnaire (HAQ-DI) (P < 0.009), and the vitality (P < 0.007) and health transition components (P < 0.003) of the short form (SF)-36 (Table 1) [12,13]. Adverse effects were seen more frequently in the CYC patients (four per CYC patient and three per PBO patient; P = 0.01).…”