Table 4 Clinical-biological characteristics and therapies administered in patients with fasciitis (n = 39)
From: Graft versus host disease-related eosinophilic fasciitis: cohort description and literature review
Variables | N (%) |
|---|---|
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%) |
Superficial scleroderma | 2 (5.1%) |
Deep scleroderma | 33 (84.6%) |
Mixed (scleroderma/lichenoid) | 13 (33.3%) |
Combined scleroderma | 18 (46.2%) |
Biopsy information | 22 (56.4%) |
Lichenoid | 7 (18%) |
Deep Sclerodermiform | 10 (25.6%) |
Mixed (scleroderm/lichenoid) | 4 (10.2%) |
Fasciitis | 1 (2.5%) |
Thrombopenia at diagnosis (< 100,000/μL) | 1 (2.6%) |
Eosinophilia at diagnosis (> 500/mm3) | 21 (53.8%) |
Positive autoantibodies | 10 (25.7%) |
Synovial fluid study (inflammatory) | 2 (5.1%) |
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 (94.9%) |
Rescue treatment | |
Extracorporeal photopheresis | 25 (64.1%) |
Ruxolitinib | 8 (20.5%) |
Imatinib | 10 (25.6%) |
Others | 11 (28.2%) |
Physiotherapy program | 15 (38.5%) |
Best response achieved | |
Complete response | 16 (41.0%) |
Partial response | 20 (51.3%) |
Refractoriness | 2 (5.1%) |
PGA* < 4 | 22 (56.4%) |
PhGA$ < 4 | 22 (56.4%) |
Exitus | 7 (17.9%) |
Relapse | 2 |
cGVHD progression | 1 |
Infection | 2 |
Other | 2 |