Table 2

Incidence of progressive disease in SSc according to SSc subtype Progressive disease, N (%)

TotalDcSScLcSScLSSc
N=171N=64N=79N=28
Deceased8 (5)3 (5)4 (5)1 (4)
≥10% decrease in body weight10 (6)5 (8)2 (3)3 (11)
≥30% increase with minimum of Δ5 in MRSS*15 (9)7 (11)7 (9)1 (4)
≥30% decrease in eGFR6 (4)3 (5)3 (4)0 (0)
≥10% decrease in FVC*13 (8)3 (5)6 (8)4 (14)
≥15% decrease in DLCO*12 (7)5 (8)6 (8)1 (4)
≥0.25 increase in SHAQ†11 (6)3 (5)8 (10)0 (0)
Total patients with progressive disease63 (37)25 (39)30 (38)8 (29)
  • *<5% missing.

  • †<15% missing.

  • DcSSc, diffuse cutaneous systemic sclerosis; DLCO, diffusing capacity for carbon monoxide; eGFR, estimated glomerular filtration rate; FVC, forced vital capacity; LcSSc, limited cutaneous systemic sclerosis; MRSS, modified Rodnan Skin Score; SHAQ, Scleroderma Health Assessment Questionnaire.