Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study

Abstract

ObjectivesOur aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features.MethodsPatients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement. Correlations between 12-month changes were also examined.ResultsThe 326 patients recruited (median disease duration 11.9 months) displayed high levels of disability [mean (s.d.) HAQ-DI 1.1 (0.83)], with ‘grip’ and ‘activity’ being most affected. Of the 18 activities assessed in the CHFS, those involving fine finger movements were most affected. High HAQ-DI and CHFS scores were both associated with high mRSS (ρ = 0.34, P < 0.0001 and ρ = 0.35, P < 0.0001, respectively). HAQ-DI was higher in patients with digital ulcers (P = 0.004), pulmonary fibrosis (P = 0.005), cardiac (P = 0.005) and muscle involvement (P = 0.002). As anticipated, HAQ-DI, CHFS, the Functional Assessment of Chronic Illness Therapy and SF36 scores were all highly correlated, in particular the HAQ-DI with the CHFS (ρ = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (ρ = 0.40, P < 0.0001), decreasing hand function (ρ = 0.57, P < 0.0001) and increasing fatigue (ρ = −0.53, P < 0.0001).ConclusionThe European Scleroderma Observational Study highlights the burden of disability in early dcSSc, with high levels of disability and fatigue, associating with the degree of skin thickening (mRSS). Impaired hand function is a major contributor to overall disability.

A patient‑centered approach to the burden of symptoms in patients with scleroderma treated with Bosentan: A prospective single‑center observational study

“…The latter were more likely to have dyspnea, greater HAQ-DI scores at baseline (P=0.051) and more severe capillaroscopic changes. Recent studies have reported more severe organ manifestations, higher mortality rates in patients with dcSSc compared to lcSSc (45,46). However, the post-treatment disability index scores were not substantially different with respect to disease phenotype in our cohort.…”

A randomised, double-blind, placebo-controlled, 24-week, phase II, proof-of-concept study of romilkimab (SAR156597) in early diffuse cutaneous systemic sclerosis

“…Secondary efficacy endpoints included change from baseline to week 24 in the Health Assessment Questionnaire-Disability Index (HAQ-DI), a measure of physical/functional disability, assessed using the Scleroderma Health Assessment Questionnaire (SHAQ) [19][20][21] and observed FVC/observed diffusing lung capacity for carbon monoxide (DL CO ; corrected for haemoglobin).…”

Impact of EUSTAR standardized training on accuracy of modified Rodnan skin score in patients with systemic sclerosis

“…The extent and degree of skin thickness are useful surrogate markers of SSc disease activity. Previous studies demonstrated associations between more extensive skin involvement and severe organ damage, disability, and poor prognosis . Notably, previous research showed that patients with rapid skin thickness progression were at increased risk of mortality and renal crisis .…”