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%) |