Table 4.
Comparison between lower ELVAR group and higher ELVAR group.
| Variables | MDA5 + DM patients | ||
|---|---|---|---|
| lower ELVAR group N = 24 | higher ELVAR group N = 31 | P-value | |
| Female, n (%) | 20(83.3) | 22(71.0) | 0.289 |
| Age of onset (y) | 53(46.25, 59.25) | 52(42, 59) | 0.440 |
| Duration from onset to treatment, m | 3.0(2.0, 4.0) | 2.0(1.0, 3.0) | 0.095 |
| Duration from treatment to death or 1 year, m | 2.25(0.5, 12.0) | 12.0(12.0, 12.0) | 0.000* |
| Mortality within 3 months, n (%) | 13(54.2) | 3(9.7) | 0.000* |
| Mortality within 12 months, n (%) | 13(54.2) | 4(12.9) | 0.001* |
| Smoking history, n (%) | 3(12.5) | 5(16.1) | 0.708 |
| Clinical feature | |||
| Muscle weakness, n (%) | 12(50.0) | 21(67.7) | 0.187 |
| Gottron sign, n (%) | 7(29.2) | 7(22.6) | 0.582 |
| Heliotrop rash, n (%) | 8(33.3) | 11(35.5) | 0.869 |
| Skin ulcer, n (%) | 6(25.0) | 8(25.8) | 0.946 |
| Raynaud phenomenon, n (%) | 0(0.0) | 3(9.7) | 0.120 |
| Fever, n (%) | 8(33.3) | 7(22.6) | 0.379 |
| Cough, n (%) | 4(16.7) | 7(22.6) | 0.590 |
| Dyspnea, n (%) | 19(79.2) | 15(48.4) | 0.021* |
| Pneumothorax, n (%) | 1(4.1) | 1(3.2) | 0.855 |
| Concomitant neoplasia, n (%) | 0(0.0) | 1(2.6) | 0.379 |
| Laboratory data | |||
| Neutrophil Count, X109/L | 3.31(2.67, 4.74) | 3.29(2.37, 4.63) | 0.754 |
| Lymphocyte Count, X109/L | 0.65(0.50, 0.87) | 0.92(0.63, 1.34) | 0.022* |
| ESR, mm/hr | 43(31, 62) | 32.50(24.50, 56.25) | 0.164 |
| CRP, mg/L | 9.21(2.60, 18.03) | 5.16(2.60, 11.10) | 0.222 |
| CK, IU/L | 76.20(45.83, 144.23) | 87.4(44.6, 201.2) | 0.905 |
| LDH, IU/L | 378.60(332.80, 525.55) | 358.50(268.25, 441.05) | 0.157 |
| Myoglobin, ug/L | 69.60(49.50, 108.78) | 69.4(38.5, 95.2) | 0.457 |
| Albumin, g/L | 27.40(24.33, 30.83) | 29.8(26.7, 34.2) | 0.051 |
| Ferritin, ng/ml | 519.05(375.45, 570.19) | 485.18(357.90, 562.67) | 0.131 |
| PCT, ng/ml | 0.09(0.053, 0.14) | 0.07(0.035, 0.11) | 0.214 |
| Pulmonary involvement | |||
| ILD diagnosis, n (%)& | 22(91.7) | 26(83.9) | 0.394 |
| Radiological feature of ILD at diagnosis | |||
| Reticulations, n (%) | 6(25.0) | 8(25.8) | 0.946 |
| Septal thickening, n (%) | 8(33.3) | 11(35.5) | 0.869 |
| Ground glass opacities, n (%) | 7(29.2) | 6(19.4) | 0.400 |
| Nodules, n (%) | 3(12.5) | 9(29.0) | 0.145 |
| Bronchiectasis/Bronchiolectasis, n (%) | 1(4.2) | 2(6.5) | 0.714 |
| Honeycombing, n (%) | 0(0.0) | 2(6.5) | 0.209 |
| Consolidation, n (%) | 18(75.0) | 16(51.6) | 0.079 |
| ELVAR | 0.7893(0.7442,0.8143) | 0.8823(0.8638,0.9025) | 0.000* |
| Severity of type I respiratory failure | |||
| no/mild/moderate/severe, n (%) | 11/4/7/2 (45.8/16.7/29.2/8.3) | 24/4/3/0 (77.4/12.9/9.7/0) | 0.009* |
| no, n (%) | 11(45.8) | 24(77.4) | 0.017* |
| mild, n (%) | 4(16.7) | 4(12.9) | 0.697 |
| moderate, n (%) | 7(29.2) | 3(9.7) | 0.066 |
| severe, n (%) | 2(8.3) | 0(0) | 0.105 |
| P/F ratio, mmHg | 260(192.5, 419) | 417(303, 471) | 0.025* |
| Oxygen supplementation | |||
| 0/1–2/3–4/>4L/min, n (%) | 5/5/7/7 (20.8/20.8/29.2/29.2) | 17/4/8/2 (54.8/12.9/25.8/6.5) | 0.007* |
| 0L/min, n (%) | 5(20.8) | 17(54.8) | 0.011* |
| 1–2L/min, n (%) | 5(20.8) | 4(12.9) | 0.435 |
| 3–4L/min, n (%) | 7(29.2) | 8(25.8) | 0.783 |
| >4L/min, n (%) | 7(29.2) | 2(6.5) | 0.025* |
| FVC, % PRED | 79(71,82) | 79(78,81) | 0.843 |
| DLCO, % PRED | 79(77.25,79.75) | 79(68.50,80.25) | 0.902 |
| Antibody positive | |||
| ANA, n (%) | 7(29.2) | 10(32.3) | 0.807 |
| MAA profile | 19(79.2) | 22(71.0) | 0.493 |
| SSA, n (%) | 2(8.3) | 6(19.4) | 0.255 |
| SSB, n (%) | 2(8.3) | 1(3.2) | 0.412 |
| Ro-52, n (%) | 18(75.0) | 21(67.7) | 0.560 |
| CENP-B, n (%) | 1(4.2) | 1(3.2) | 0.855 |
| Ku, n (%) | 0(0.0) | 1(3.2) | 0.379 |
| PM-Scl, n (%) | 2(8.3) | 0(0.0) | 0.105 |
| MSA profile, n (%) | 7(29.2) | 6(19.4) | 0.400 |
| anti-ARS antibodies, n (%) | 2(8.3) | 3(9.7) | 0.865 |
| Jo-1, n (%) | 0(0.0) | 2(6.5) | 0.209 |
| PL-7, n (%) | 0(0.0) | 1(3.2) | 0.379 |
| PL-12, n (%) | 1(4.2) | 1(3.2) | 0.855 |
| EJ, n (%) | 0(0.0) | 0(0.0) | 1.000 |
| OJ, n (%) | 1(4.2) | 0(0.0) | 0.256 |
| Non-anti-ARS antibodies, n (%) | 5(20.8) | 5(16.1) | 0.657 |
| SRP, n (%) | 1(4.2) | 1(3.2) | 0.855 |
| TIF1-γ, n (%) | 1(4.2) | 2(6.5) | 0.714 |
| Mi-2β, n (%) | 2(8.3) | 3(9.7) | 0.865 |
| SAE, n (%) | 1(4.2) | 0(0.0) | 0.256 |
| Treatment | |||
| Glucocorticoid, <1 /≥1 mg/kg/d, n (%) | 12/12(50.0/50.0) | 16/15(51.6/48.4) | 0.906 |
| Methotrexate, n (%) | 2(8.3) | 3(9.7) | 0.865 |
| Cyclophosphamide, n (%) | 19(79.2) | 20(64.5) | 0.240 |
| Cyclosporine A, n (%) | 1(4.2) | 7(22.6) | 0.057 |
| Tacrolimus, n (%) | 2(8.3) | 5(16.1) | 0.394 |
| Plasma exchange, n (%) | 4(16.7) | 3(9.7) | 0.445 |
| Single drug or Combination drugs | |||
| 1 drug | 3(12.5) | 5(16.1) | 0.708 |
| 2 drugs | 15(62.5) | 16(51.6) | 0.424 |
| 3 drugs | 5(20.8) | 8(25.8) | 0.670 |
| ≥ 4 drugs | 1(4.2) | 2(6.5) | 0.714 |
*
P < 0.05.
&
ILD diagnosis by a radiologist and a respirologist simultaneous.
ANA, antinuclear antibody; CRP, C reactive protein; CK, creatine kinase; DLCO, carbon monoxide diffusing capacity; ELVAR, Efficient Lung Vetilation Area Ratio; ESR, erythrocyte sedimentation rate; FVC, forced vital capacity; ILD, interstitial lung disease; LDH, Lactic dehydrogenase; MDA5, melanoma melanoma differentiation-associated gene-5; PCT, procalcitonin; P/F ratio, PaO2/FiO2 ratio; SAE, anti-small ubiquitin-like modifier antibody; SRP, signal recognition particle; TIF1-γ, transcription intermediary factor 1-gamma.