Lung function is associated with minimal EQ-5D changes over time in patients with systemic sclerosis

Abstract

In systemic sclerosis (SSc) therapeutic efforts are often directed to prevent progressive respiratory impairment, but it is unclear to what extent changes in pulmonary function tests (PFTs) are associated with health-related quality of life (HRQoL). The aim of our study is to evaluate how modifications in PFTs contribute to longitudinal variations in HRQoL, assessed through the multidimensional questionnaire EQ-5D, in patients with SSc. We included SSc patients with forced vital capacity (FVC%), diffusing capacity of the lungs for carbon monoxide (DLCO%) and EQ-5D assessed in at least two visits. The EQ-5D consists of two parts, a utility score ranging from − 0.59 to 1, and a 0-100 Visual Analogue Scale (VAS). Higher values represent better health. The association between changes in FVC% and DLCO%, and evolution of EQ-5D over time, was investigated using generalized estimating equations. Three hundred seventy-eight patients were included, accounting for a total of 1619 measurements. The models showed that improvement in FVC% is significantly associated with increase in both utility score (β = 0.001; 95% CI 0.000 to 0.002; p = 0.003) and VAS over time (β = 0.188; 95% CI 0.111 to 0.264; p < 0.001). Moreover, improvement in DLCO% is longitudinally associated with increase in utility score (β = 0.001; 95% CI 0.000 to 0.002; p = 0.038), while the results for VAS were non-significant (β = 0.020; 95% CI-0.079 to 0.120; p = 0.690). We show that change in PFTs has a significant, although minor, impact on HRQoL as measured by EQ-5D in SSc. Key Points • In patients with SSc, changes in PFTs have a significant, although minor, impact on HRQoL. • In patients with SSc-ILD, the perception of HRQoL is nearly not influenced by changes in pulmonary function. • The use of generic questionnaires might not be sensitive enough to evaluate the impact on quality of life of therapies targeting specific SSc manifestations.

Quality of life in SSc-ILD patients: Understanding the impact of the ILD and the needs of the SSc-ILD patients and their need for caregivers in France

“…30 In our study, the EQ-5D-5L score increased significantly by 0.03 for 10% increase of the FVC. This finding is consistent with that of Ciaffi et al, 31 who had performed a retrospective study in 378 patients with SSc including 156 patients with ILD and pulmonary arterial hypertension. They reported that changes in lung function have a significant impact on HRQoL using the EQ-5D score in both its utility score (p = 0.003) and VAS (p < 0.001) over time.…”

Untangling the relationship between smoking and systemic sclerosis: an analysis of the EUSTAR cohort

“…In accordance with earlier literature, our study shows that a history of smoking is associated with a lower prevalence of ATA 10 23 and we confirm that this imbalance is found typically in female patients. 24 Additionally, we notice that this association is dose-dependent (ie, the higher the exposure rate, the lower the chance to be ATApositive), which underlines the hypothesis that, indeed, exposure to cigarette smoke might impact the ATAspecific B cell responses. This finding is unexpected if we consider what happens in other autoimmune diseases such as RA or systemic lupus erythematosus (SLE).…”

“…10 23 In an analysis of the autoantibody profile of 361 patients of the Leiden Combined Care in SSc cohort, we reported the same finding, but we also showed that smoking might have different effects on autoantibodies in men and women, with never-smoking women being more frequently ATApositive than ever-smoking women. 24 In rheumatoid arthritis (RA), smoking has a wellestablished impact on disease susceptibility and confers a risk for the development of anti-citrullinated peptide antibodies (ACPA) and rheumatoid factor, in particular in patients carrying specific HLA-DRB1 variants. 25 These observations have fuelled the hypothesis that, in a susceptible individual, chronic exposure to specific antigens, for example, in the lungs, can trigger a targeted chronic autoimmune response.…”

Insights into origins and specificities of autoantibodies in systemic sclerosis

“…Separate case studies from Italy confirmed a higher frequency of silicone breast implants [13] and a higher risk for rupture [14] specifically in patients with ARA-positive SSc. Although smoking has been identified as a potential trigger for autoimmunity in general [15], and a risk factor for worse prognosis in patients with SSc [16], ATA were found to be negatively associated with smoking (OR 1.77, 95% CI 1.04-2.99, P ¼ 0.034) [17]. This is an unexpected finding of a detrimental environmental factor acting as a protective influence against developing specific autoantibodies in SSc and merits further evaluation.…”