Table 2 Proposed items for future research
Research agendaMedian (IQR)
1Measure of telangiectasia5 (3–7)
2Visual analogue scale (VAS) or/Likert patient global assessment for pruritus7 (5–8)
1Large joint contracture7 (4–8)
2Michigan Hand Questionnaire187 (6–8)
1Serum pro-brain natriuretic peptide (Pro-BNP) or NT-Pro-BNP7 (6–8)
2Non-invasive measures of cardiac function, eg, cardiac magnetic resonance imaging, tissue Doppler7 (6–8)
1Scleroderma gastrointestinal 1.0 questionnaire38 (7–9)
2Gastric emptying time and/or 24-h small bowel transit time7 (6–8)
Health-related quality of life and function:
1Measure of health utility eg, Short Form-6D (SF-6D), EuroQol, Quality of Well Being Scale, time trade-off, standard gamble7 (7–9)
2Measure of fatigue, eg, functional assessment of chronic illness therapy (FACIT)-fatigue7 (5–8)
3Measure of depression/anxiety, eg, Beck Depression Inventory, Center for Epidemiologic Depression Scale6 (5–8)
Global health:
1Medsger Severity Index297 (5–8)
Digital ulcers
1Development of digital ulcer condition score that captures activity, severity, and impact7 (6.5–9)
1Markers of the collagen breakdown, eg, soluble interleukin (IL)2 receptor levels, procollagen I and III aminopropeptide, connective tissue growth factor (CTGF) levels, serum collagen I carboxyterminal telopeptide, urinary pyridinoline cross-link compounds of collagen7 (5–8)
  • IQR, interquartile range.