Criteria | Whole series | dcSSc | lcSSc |
---|---|---|---|
*Kahaleh total skin score4; †deterioration, as evaluated by the patient, of the condition in the month preceding the assessment. | |||
TTS, total skin score; Tlco, carbon monoxide transfer factor; ESR, erythrocyte sedimentation rate. | |||
TTS* >20 | 1.0 | ||
Scleredema | 0.5 | 0.5 | |
Δ Skin† | 2.0 | 3.0 | 2.5 |
Digital necrosis | 0.5 | ||
Δ Vascular† | 0.5 | 2.0 | 1.0 |
Arthritis | 0.5 | 1.0 | |
Δ Articular/muscular† | 1.0 | ||
↓ Tlco (<80% of predicted value) | 0.5 | ||
Δ Cardiopulmonary† | 2.0 | 4.0 | 1.5 |
ESR >30 mm/1st h | 1.5 | 2.5 | |
Hypocomplementaemia (C3 and/or C4) | 1.0 | 1.0 | |
Total maximum disease activity index | 10.0 | 10.0 | 10.0 |