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. 2022 Nov 2;57(Suppl 1):100–416. doi: 10.1038/s41409-022-01798-0
Nonspecific prodromal symptoms
• Absent 8 (20.5%)
• Stiffness 33 (84.6%)
• Arthromyalgias 24 (61.3%)
• Edemas 11 (28.2%)
• Cramps 9 (23.0%)
• Skin tightness 29 (74.4%)
Joint contracture 14 (35.8%)
Affected range of motion (ROM):Mild/moderate / severe 23 (59.0%)/10 (25.6%)/1 (2.6%)
Limitation of upper limb mobility:
• P-ROM shoulders 23 (58.9%)
• P-ROM elbows 13 (33.4%)
• P-ROM wrists/fingers 19 (50.0%)
Limitation of mobility of lower limbs:
• P-ROM ankles 14 (35.9%)
Concomitant skin sclerosis 35 (89.7%)
Eosinophilia at diagnosis (>500/mm3) 21 (53.8%)
Positive autoantibodies 10 (25.7%)

Imaging tests performed:

• Rx/MRI / Echo

2 (5.1%)/2 (5.1%)/3 (7.7%)
Median number of treatment lines (range) 3 (1-7)
First-line treatment
• Corticosteroids 37 (94.4%)
Rescue treatment
• Extracorporeal photopheresis 25 (64.1%)
• Ruxolitinib 8 (20.5%)
• Imatinib 10 (25.6%)
• Others 11 (28.2%)