Table 3.
Disease outcomes and medications used in JDM patients with and without anti-HSC70 autoantibodies
| Disease outcomes and medications, ever present | Anti-HSC70 positive (N = 22) | Anti-HSC70 negative (N = 41) | P-value |
|---|---|---|---|
| Ever hospitalized, n/N (%) | 17/22 (77) | 24/41 (59) | 0.2 |
| Median number of hospitalizations, median (IQR) | 2.0 (0.5–4.5) (N = 19) | 1.0 (0–1.5) (N = 39) | <0.04 |
| Wheelchair/devicea use, n/N (%) | 14/22 (64) | 11/41 (27) | <0.007 |
| Wheelchair use, n/N (%) | 12/22 (55)c | 9/41 (22)c | <0.02 |
| 4/22 (18)d | 1/41 (2)d | <0.05 | |
| Disease activity at final assessment, median (IQR), median (IQR) | 1.0 (1.0–2.0) | 1.0 (1.0–1.0) | 0.1 |
| Physician global activity scoreb, median (IQR) | 2.3 (1.5–3.9) | 2.5 (1.7–3.5) | 0.7 |
| Physician global damage assessmentb, median (IQR) | 2.2 (1.0–3.8) (N = 21) | 1.4 (0.7–2.7) (N = 30) | 0.07 |
| Myositis Damage Index severity of damage scoreb, median (IQR) | 8.0 (5.0–14.0) (N = 21) | 5.0 (2.0–7.0) (N = 29) | 0.01 |
| CHAQ scoreb, median (IQR) | 2.1 (1.0–4.7) | 2.6 (1.1–4.7) | 0.6 |
| MMT8 score (left)b, median (IQR) | 74.0 (66.0–80.0) (N = 21) | 72.5 (66.5–77.3) (N = 32) | 0.6 |
| MMT8 score (right)b, median (IQR) | 74.0 (66.0–80.0) (N = 21) | 75.0 (66.5–78.0) (N = 32) | 0.9 |
| CMAS scoreb, median (IQR) | 42.5 (34.8–48.3) (N = 20) | 44.0 (38.3–49.0) (N = 30) | 0.4 |
| Disease course, n/N (%) | |||
| Monocyclic course | 0/16 (0) | 2/27 (7) | 0.5 |
| Polycyclic course | 2/16 (13) | 3/27 (11) | 1.0 |
| Chronic continuous course | 14/16 (88) | 22/27 (81) | 0.7 |
| Response to treatment | |||
| Complete clinical response, n/N (%) | 1/22 (5) | 7/40 (18) | 0.2 |
| Remission, n/N (%) | 0/22 (0) | 1/40 (3) | 1.0 |
| Prednisone discontinuatione, n/N (%) | 0/22 (0) | 3/40 (8) | 0.5 |
| Total number of medications used, median (IQR) | 7.0 (5.3–8.0) (N = 22) | 6.0 (4.0–7.3) (N = 40) | 0.2 |
| Medications received, n/N (%) | |||
| Oral steroids | 22/22 (100) | 40/40 (100) | 1.0 |
| Intravenous pulsed steroids | 20/22 (91) | 30/40 (75) | 0.2 |
| Methotrexate | 21/22 (95) | 39/40 (98) | 1.0 |
| Intravenous immunoglobulin | 21/22 (95) | 29/40 (73) | <0.05 |
| Other DMARDs | 22/22 (100) | 39/40 (98) | 1.0 |
Categorical variables are analysed by Fisher's exact tests and are presented as count/total (percentage). Continuous variables are analysed by the Mann–Whitney U test and are expressed as median (IQR [Q1–Q3]).
a
Wheelchair, bed bound and other assistive devices are included.
b
Evaluation at the time of measurement of anti-HSC70 autoantibodies.
c
In the observation period.
d
At last follow-up.
e
Discontinuation of prednisone in first 12 months of treatment.
CHAQ: childhood HAQ; IQR: interquartile range; CMAS: Childhood Myositis Assessment Scale.