Table 1.

Clinical characteristics at time of enrolment in the ItinérAIR-Sclérodermie registry according to systemic sclerosis subtype. Categorical data are expressed as percentages, continuous data are mean ± SD.

AlldcSSclcSScp
Patients, n599165434
Male, %1622140.01
Age, yrs55 ± 1353 ± 1355 ± 130.10
Occupational risks*, %1113100.23
Delay between RP onset and first non-RP clinical symptom, yrs6 ± 93 ± 67 ± 10< 0.0001
Time since first non-RP symptom onset, yrs7 ± 78 ± 77 ± 70.09
Age at first non-RP symptom, yrs46 ± 1444 ± 1447 ± 140.008
Never smoker, %6867690.64
Current smoker, %1215110.24
Tobacco consumption (pack/yr)5 ± 126 ± 165 ± 100.18
Rodnan score13 ± 1126 ± 129 ± 5< 0.0001
Digital sclerosis score7 ± 39 ± 36 ± 3< 0.0001
TLC < 80% predicted, %152312< 0.0001
FVC < 80% predicted, %13248< 0.0001
DLCO < 60% predicted, %243919< 0.0001
  • * Exposure to silicates, solvents, glue, or resin.

  • Never-smokers assigned a consumption of zero. RP: Raynaud’s phenomenon; dcSSc: diffuse cutaneous systemic sclerosis; DLCO: lung diffusing capacity for carbon monoxide; lcSSc: limited cutaneous systemic sclerosis; TLC: total lung capacity; FVC: forced vital capacity.