Table 1.
Comparison of clinical characteristics between severe-ILD group and non-severe ILD group in MDA5+DM-ILD.
| Characteristic | Overall cohort (N = 51) | Severe-ILD group (n = 24) | Non-severe-ILD group (n = 27) | p Value |
|---|---|---|---|---|
| Demographic | ||||
| Age of onset, years | 45.45 ± 13.03 | 50.17 ± 2.42 | 41.26 ± 2.46 | .013* |
| Female, n (%) | 35 (68.63) | 15 (62.50) | 20 (74.07) | .374 |
| Height, cm | 160.02 ± 6.91 | 160.71 ± 6.84 | 159.10 ± 7.38 | .569 |
| Weight, kg | 54.67 ± 11.21 | 57.09 ± 11.04 | 53.21 ± 10.90 | .216 |
| Duration from onset to treatment, months | 4.24 ± 4.56 | 3.46 ± 3.48 | 4.93 ± 5.32 | .245 |
| Duration of ILD, months | 1.57 ± 2.83 | 1.625 ± 2.86 | 1.519 ± 2.86 | .895 |
| Smoker ever, n (%) | 10 (19.61) | 5 (20.83) | 5 (18.52) | 1.000 |
| Clinical features | ||||
| Fever, n (%) | 19 (37.25) | 10 (41.67) | 9 (33.33) | .539 |
| Cough, n (%) | 22 (41.14) | 12 (50.00) | 10 (37.04) | .351 |
| Dyspnoea, n (%) | 24 (47.06) | 14 (58.33) | 10 (37.04) | .128 |
| Myalgia, n (%) | 10 (19.61) | 5 (20.83) | 5 (18.52) | .714 |
| Hoarseness or sore throat, n (%) | 7 (13.73) | 5 (20.83) | 2 (7.41) | .232 |
| Mouth ulcers, n (%) | 5 (9.80) | 3 (12.50) | 2 (7.41) | .656 |
| Muscle weakness, n (%) | 19 (37.25) | 10 (41.67) | 9 (33.33) | .539 |
| Arthralgia, n (%) | 27 (51.94) | 10 (41.67) | 17 (62.96) | .128 |
| Raynaud phenomenon, n (%) | 1 (1.96) | 1 (4.17) | 0 (0.00) | .471 |
| Finger swollen, n (%) | 8 (15.69) | 5 (20.83) | 3 (11.11) | .451 |
| Mechanics hand, n (%) | 18 (35.29) | 9 (37.5) | 9 (33.33) | .756 |
| Skin ulcer, n (%) | 13 (25.49) | 9 (37.5) | 4 (14.81) | .107 |
| Periorbital swelling, n (%) | 17 (33.33) | 11 (45.83) | 6 (22.22) | .074 |
| Heliotrope rash, n (%) | 29 (56.86) | 13 (54.17) | 16 (59.26) | .782 |
| Gottron’s sign, n (%) | 33 (64.71) | 15 (62.5) | 18 (66.67) | .756 |
| Laboratory data | ||||
| Lymphocyte count, ×109/L | 0.93 ± 0.48 | 0.87 ± 0.41 | 0.99 ± 0.54 | .396 |
| Lymphocyte ration, (%) | 16.65 ± 8.61 | 17.80 ± 9.39 | 15.35 ± 7.62 | .316 |
| ESR, mm/1 h | 42.07 ± 28.15 | 53.22 ± 32.20 | 33.35 ± 21.41 | .032* |
| CRP, mg/L | 6.29 [1.30, 16.28] | 8.87 [3.07, 26.57] | 2.890 [0.76, 7.88] | .015[U] |
| CK, IU/L | 68.50 [41.00, 230.00] | 130.00 [51.50, 527.75] | 48.500 [34.00, 163.75] | .023[U] |
| LDH, IU/L | 327.00 [249.50, 516.00] | 400.00 [331.00, 527.00] | 281.00 [232.00, 352.50] | .001[U] |
| Ferritin, ng/mL | 1171.70 [326.96, 2076.16] | 1954.50 [1308.45, 2225.48] | 471.86 [141.09, 1664.86] | .006[U] |
| PCT, ng/mL | 0.080 [0.05, 0.15] | 0.11 [0.07, 0.19] | 0.08 [0.03, 0.09] | .022[U] |
| Albumin, g/L | 32.60 ± 4.92 | 31.11 ± 3.83 | 33.98 ± 5.47 | .039* |
| ALT, U/L | 33.00 [22.00, 85.00] | 51.000 [28.00, 99.50] | 30.00 [16.00, 57.00] | .027[U] |
| AST, U/L | 43.00 [25.00, 94.00] | 76.50 [43.00, 128.25] | 28.00 [22.00, 73.00] | .001[U] |
| Cr, μmol/L | 55.92 ± 15.28 | 55.83 ± 17.70 | 56.00 ± 13.22 | .968 |
| PRO-BNP, pg/mL | 186.05 [91.95, 186.05] | 146.70 [76.93, 254.13] | 271.05 [152.75, 325.15] | .111[U] |
| P/F ration, mmHg | 336.60 ± 94.75 | 288.20 ± 77.38 | 402.28 ± 75.74 | .000** |
| D-dimer, μg/mL | 1.40 [0.78, 1.99] | 1.675 [0.82, 2.09] | 1.03 [0.73, 1.67] | .118[U] |
| IgG, g/L | 15.92 ± 6.73 | 17.97 ± 8.72 | 13.97 ± 3.18 | .066 |
| IgM, g/L | 1.46 ± 0.53 | 1.26 ± 0.51 | 1.65 ± 0.49 | .017* |
| TNF-α, pg/mL | 1.47 ± 1.20 | 1.81 ± 1.48 | 1.12 ± 0.76 | .183 |
| IFN-γ, pg/mL | 1.48 [0.78, 1.99] | 0.90 [0.68, 2.07] | 1.65 [0.40, 2.32] | .818[U] |
| IL-4, pg/mL | 1.11 ± 0.70 | 1.52 ± 0.60 | 0.71 ± 0.56 | .004** |
| IL-6, pg/mL | 6.50 [3.40, 23.21] | 9.49 [5.44, 91.90] | 4.15 [1.93, 14.02] | .073[U] |
| MDA5 titer | .872 | |||
| (+) | 16 (31.4) | 8 (33.3) | 8 (29.6) | |
| (++) | 10 (19.6) | 4 (16.7) | 6 (22.2) | |
| (+++) | 25 (49.0) | 12 (50) | 13 (48.1) | |
| Presence of Ro52, n (%) | 35 (68.63) | 22 (91.67) | 13 (48.15) | .001** |
| Ro52 titer | .004** | |||
| (+) | 7 (13.73) | 5 (20.83) | 2 (7.41) | |
| (++) | 5 (9.80) | 4 (16.67) | 1 (3.70) | |
| (+++) | 23 (45.10) | 13 (54.17) | 10 (37.04) | |
| Pulmonary function test | ||||
| FVC, %pred | 75.48 ± 16.48 | 71.10 ± 18.58 | 77.91 ± 15.20 | .304 |
| DLCO, %pred | 57.23 ± 11.32 | 51.59 ± 11.37 | 60.27 ± 10.48 | .103 |
| FEV1, %pred | 74.35 ± 15.02 | 72.51 ± 19.88 | 75.37 ± 12.10 | .685 |
| Type of ILD at diagnosis | ||||
| OP/OP + NSIP, n (%) | 34 (66.67) | 15 (62.50) | 19 (70.37) | .552 |
| NSIP, n (%) | 17 (33.33) | 9 (37.50) | 8 (29.63) | .552 |
| UIP, n (%) | 0 (0.00) | 0 (0.00) | 0 (0.00) | – |
| Initial treatment | ||||
| Glucocorticoid dose | ||||
| ≤1 mg/kg/d, n (%) | 42 (82.35) | 18 (75.00) | 24 (88.89) | .276 |
| >1 mg/kg/d, n (%) | 9 (17.64) | 6 (25.00) | 3 (11.11) | .276 |
| Pulse therapy, n (%) | 8 (15.69) | 5 (20.83) | 3 (11.11) | .451 |
| CXT, n (%) | 17 (33.33) | 11 (45.83) | 6 (22.22) | .074 |
| CsA, n (%) | 5 (9.80) | 4 (16.67) | 1 (3.70) | .175 |
| Tacrolimus, n (%) | 13 (25.49) | 5 (20.83) | 8 (29.63) | .472 |
| MMF, n (%) | 4 (7.84) | 0 (0.00) | 4 (14.81) | .113 |
| JAK, n (%) | 13 (25.49) | 6 (25.00) | 7 (25.93) | 1.000 |
| IVIG, n (%) | 18 (35.29) | 11 (45.83) | 7 (25.93) | .138 |
| Anti-fibrosis therapy, n (%) | 17 (33.33) | 7 (29.17) | 10 (37.04) | .552 |
| Combination therapy | ||||
| Glucocorticoid + CTX + CNI, n (%) | 9 (17.65) | 5 (20.83) | 4 (14.81) | .718 |
| Glucocorticoid + IS + JAK, n (%) | 9 (17.65) | 4 (16.67) | 5 (18.52) | 1.000 |
[U]: results of Mann–Whitney’s U-test; ALB: albumin; ALT: alanine transaminase; AST: aspartate transaminase; CK: creatine kinase; Cr: creatinine; CRP: C-reactive protein; CNI: calcineurin inhibitor (cyclosporine or tacrolimus); CsA: cyclosporine A; CTX: cyclophosphamide; DLCO: carbon monoxide diffusion capacity of the lung; ESR: erythrocyte sedimentation rate; FEV1: forced expiratory volume in 1 s; FVC: forced vital capacity ; IFN-γ: interferon-γ; IL-4/6: interleukin-4/6; IS: immunosuppressant drugs, including cyclophosphamide, cyclosporine, tacrolimus, mycophenolate mofetil; IVIG: intravenous immunoglobulin; JAK inhibitor: Janus kinase inhibitor; LDH: lactic dehydrogenase; MDA5: anti-melanoma differentiation-associated gene 5 antibody; MMF: mycophenolate mofetil; MSAs: myositis-specific autoantibodies; NSIP: nonspecific interstitial pneumonia; OP: organic pneumonia; P/F ration: arterial oxygen/fraction of inspiration oxygen; PCT: procalcitonin; pro-BNP: pro-brain natriuretic peptide; Ro52: anti-Ro52 antibody; TNF-α: tumour necrosis factor-α; UIP: usual interstitial pneumonia.
Values are expressed as mean ± standard deviation, median (IQR) or n (%).
p < .05.
p < .01.