Disease duration of scleroderma(1st non-Raynaud's) |
|
At the time of muscle biopsy (years) |
6.0 ± 7.0 |
At the onset of muscle weakness (years) |
4.2 ± 7.5 |
Disease duration of scleroderma (Raynaud's) |
At the time of muscle biopsy (years) |
9.4 ± 18.3 |
At the onset of muscle weakness (years) |
7.9 ± 19.3 |
Lab values |
Elevated maximum CK (CK≥200) (31/42, 73.8%) |
2412 ± 3317 |
Normal maximum CK (CK<200) (11/42, 26.2%) |
94.4 ± 56.1 |
MRI Findings N=34 |
MRI edema |
30 (88.2%) |
MRI fatty replacement |
10 (29.4%) |
EMG results N=39 |
Irritable myopathy |
19 (48.7%) |
Non-irritable myopathy |
16 (41.3%) |
Normal |
4 (10.3%) |
Nerve Conduction Results N=39 |
Abnormal sural study |
17 (43.5%) |
Muscle biopsy features |
Inflammation (N=42) |
20 (47.6%) |
Necrosis (N=42) |
28 (66.7%) |
Fibrosis (N=42) |
14 (33.3%) |
Acute neurogenic atrophy (N=41) |
20 (47.6%) |
Histological categories based on aggregate biopsy features |
Polymyositis |
2 (4.8%) |
Dermatomyositis |
3 (7.1%) |
Non-specific myositis |
15 (35.7%) |
Fibrosis only |
3 (7.1%) |
Necrotizing |
9 (21.4%) |
Acute denervation |
3 (7.1%) |
Other |
7 (16.6%) |