Table 3.
Summary of univariable Cox regression analyses relating risk of death and illness features
| Illness Feature* | No. of Deaths (%)
|
Hazard Ratio [95% confidence interval] | P | |
|---|---|---|---|---|
| Illness Feature Absent | Illness Feature Present | |||
| Clinical subgroup JDM vs. JCTM | JDM 8/329 (2.4%) | JCTM 7/46 (15.2%) | 9.8 [3.1, 31.4] | <0.001 |
| Clinical subgroup JDM vs. JPM | JPM 2/30 (6.7%) | 7.0 [1.3, 37.7] | 0.03 | |
| Illness severity at onset (mild/moderate vs. severe/very severe) | 7/278 (2.5%) | 10/127 (7.9%) | 2.9 [1.1, 7.6] | 0.03 |
| Age at diagnosis (years)† | 7.4 [5.1, 11.6] | 14.7 [9.0, 16.4] | 1.2 [1.1, 1.3] | <0.001 |
| Age at onset (years)† | 6.9 [4.5, 11.0] | 12.3 [8.5, 15.0] | 1.2 [1.1, 1.3] | 0.002 |
| Delay to diagnosis (months)† | 4.0 [2.0, 9.0] | 6.0 [2.0, 12.7] | 1.02 [1.0, 1.0] | 0.03 |
| Clinical Features Present Prior to or at Diagnosis | ||||
| Dysphagia‡ | 14/389 (3.6%) | 1/1 (100%) | 375.5 [23.5, 6003.2] | <0.001 |
| Abdominal perforati on‡ | 16/403 (4.0%) | 1/1 (100%) | 39.4 [4.8, 325.2] | 0.001 |
| Ever hospitalized | 1/183 (0.6%) | 15/208 (7.2%) | 10.5 [1.4, 80.1] | 0.02 |
| Interstitial lung disease | 12/385 (3.1%) | 3/16 (18.8%) | 9.8 [2.6, 37.1] | 0.001 |
| Raynaud’s phenomenon | 9/366 (2.5%) | 7/31 (22.6%) | 8.8 [3.2, 24.5] | <0.001 |
| Gastroesophageal regurgitation | 12/363 (3.3%) | 3/31 (9.7%) | 4.3 [1.2, 15.9] | 0.03 |
| Dysphonia | 9/324 (2.8%) | 6/64 (9.4%) | 4.1 [1.4, 11.9] | 0.009 |
| “Shawl sign” rash | 10/351 (2.9%) | 5/36 (13.9%) | 3.8 [1.3, 11.5] | 0.02 |
| Weight loss | 7/270 (2.6%) | 8/118 (6.8%) | 2.9 [1.03, 8.0] | 0.04 |
| Abdominal pain | 11/313 (3.5%) | 5/75 (6.7%) | 2.9 [0.9, 8.9] | 0.07 |
| Gottron’s papules | 7/85 (8.2%) | 8/306 (2.6%) | 0.3 [0.09, 0.8] | 0.01 |
| Myositis Autoantibodies | ||||
| Anti-Alanyl-tRNA synthetase Abs (anti-PL-12) | 15/394 (3.8%) | 2/5 (40%) | 23.6 [4.7, 117.3] | <0.001 |
| Anti-Ku Abs | 16/397 (4.0%) | 1/2 (50%) | 18.3 [2.2, 147.9] | 0.006 |
| Any aminoacyl tRNA synthetase Abs | 14/382 (3.7%) | 3/17 (17.7%) | 13.1 [3.4, 50.4] | <0.001 |
| Anti-La Abs | 16/396 (4.0%) | 1/3 (33.3%) | 12.2 [1.5, 96.5] | 0.02 |
| Anti-Sm Abs | 16/393 (4.1%) | 1/6 (16.7%) | 7.2 [0.9, 56.2] | 0.06 |
Abbreviations. JDM = juvenile dermatomyositis; JPM = juvenile polymyositis; JCTM = juvenile myositis associated with an underlying connective tissue disease; Abs=autoantibodies; PL-12=Anti-alanyl-tRNA synthetase autoantibodies.
*
All illness features with P < 0.10 included.
†
Median [25th, 75th quartiles] reported for those who did not die in the “Illness Feature Absent” column and for those who did die in the “Illness Feature Present” column.
‡
First myositis symptom reported.