Table 1.
Clinical characteristics of patients with scleroderma with fibrosis only vs. inflammation and/or necrosis on muscle biopsy
| Fibrosis only (n=8) |
Inflammation and/or
necrosis (n=29) |
p-value | |
|---|---|---|---|
| Age of scleroderma diagnosis defined by 1st non-Raynaud’s symptom | 40.1 ± 12.1 years | 45.6 ± 2.5 years | 0.35 |
| Female | 6 (75%) | 20 (69.0%) | 0.78 |
| Diffuse skin subtype | 7 (87.5%) | 18 (62.0%) | 0.18 |
| Maximum Modified Rodnan Skin Score | 23 ± 12.7 | 15.5 ±13.8 | 0.17 |
| Renal crisis | 1 (12.5%) | 5 (17.2%) | 0.75 |
| African American race | 5 (62.5%) | 11 (37.9%) | 0.20 |
| Caucasian race | 3 (37.5%) | 18 (62.7%) | |
| Disease Duration (at time of First Visit) | |||
| 1st Non-Raynaud’s Symptom | 2.31 ± 1.91 years | 2.95 ± 3.35 years | 0.49 |
| Raynaud’s Symptom | 2.01 ± 1.37 years | 3.59 ± 7.44 years | 0.31 |
| Disease duration (From Date of 1st non-Raynaud’s symptom to Muscle symptom) | 1.64 ± 1.39 years | 3.0 ± 6.36 years | 0.30 |
| Disease duration (From date of 1st symptom to muscle biopsy) | 3.96 ± 2.45 years | 4.33 ± 4.59 years | 0.73 |
| Duration of follow-up | 1.86 ± 0.53 years | 5.93 ± 4.73 years | 0.0005 |
| Mean time to death after muscle biopsy | 0.28 ± 0.19 years | 2.50 ± 1.6 years | 0.034 |
| Mean time to death from 1st non-Raynaud’s symptom | 4.9 ± 2.3 years | 10.6 ± 9.8 years | 0.34 |
| Deceased | 5 (62.5%) | 4 (14.3%) | 0.005 |
| Maximum Muscle Severity Score | 0.20 | ||
| 0 | 0 | 0 | |
| 1 | 2 (25%) | 17 (58.6%) | |
| 2 | 2 (25%) | 8 (27.6%) | |
| 3 | 1 (12.5%) | 2 (6.9%) | |
| 4 | 3 (37.5%) | 2 (6.9%) | |
| Maximum Creatine Kinase (mean) | 516 ± 391 | 2477 ± 3511 | 0.007 |
| Maximum Aldolase (mean) | 13.8 ± 4.7 | 27.3 ± 4.7 | 0.01 |
| MRI edema | 4/5 (80%) | 20/22 (95.4%) | 0.24 |
| MRI fatty replacement | 1/5 (20%) | 6/22 (27.2%) | 0.74 |
| EMG- irritable myopathy | 3/7 (42.6%) | 15/24 (62.5%) | 0.31 |
| EMG- non-irritable myopathy | 4/7 (57.1%) | 9/24 (37.5%) | |
| Minimum ejection fraction on echocardiogram | 49.7 ± 15.8 | 55.6 ± 8.5 | 0.42 |
| Minimum RVSP on echocardiogram | 44 ± 12.1 | 36.8 ± 16.0 | 0.35 |
| Minimum FVC | 55.5 ± 31.9 | 66.4 ± 17.6 | 0.23 |
| Minimum DLCO | 59.5 ± 36 | 57.3 ± 23.5 | 0.83 |
| Anti-centromere | 0/8 (0%) | 3/29 (10.3%) | 0.35 |
| Scl-70 | 3/8 (37.5%) | 2/29 (6.9%) | 0.03 |
| U1-RNP | 0/8 (0%) | 3/29(10.3%) | 0.35 |
| RNA-Poly III | 0/8 (0%) | 4/29 (13.8%) | 0.30 |
| PM-Scl | 2/7 (28.6%) | 3/27 (11.1%) | 0.25 |
| U3-RNP | 2/7 (29%) | 0/27 (0%) | 0.004 |
| Anti-Ku | 0/7 (0%) | 2/27 (7.4%) | 0.46 |
| Anti-NOR90 | 1/7 (14.3%) | 1/27 (3.7%) | 0.30 |
| Anti-Ro 52 | 0/7 (0%) | 6/27 (22.2%) | 0.18 |
| Anti-Th/To | 0/7 (0%) | 2/27 (7.4%) | 0.46 |
| ANA-nucleolar pattern | 7/7 (100%) | 9/28 (32.1%) | 0.02 |
| Improvement at 3 months in muscle strength and/or CK 3 months after biopsy | 3/8 (37.5%) | 28/29 (96.5%) | 0.001 |
| Medications 3 months after biopsy* | |||
| Prednisone (Moderate to High dose) | 1/8 (12.5%) | 22/29 (75.9%) | 0.0013 |
| Methotrexate | 2/8 (25%) | 7/29 (24%) | 0.64 |
| Mycophenalate mofetil | 4/8 (50%) | 13/29 (40.7%) | 0.55 |
| Azathioprine | 0/8 (0%) | 6/29 (20.7%) | 0.20 |
| IVIG | 1/8 (12.5%) | 4/27 (14.8%) | 0.71 |
*
Not mutually exclusive; Autoantibody data in the Table were obtained from clinical lab results (ANA, U1-RNP) or by Euroimmune assay (all other tabulated specificities). Euroimmune assay results were scored as positive when the assay values were ≥26 (that is, strong to very strong band when compared to controls).