Table 1.
Patient and disease characteristics and clinical variables stratified across the four groups
| Patients total | Group 1 No organ involvement | Group 2 Pulmonary only | Group 3 cardiac only | Group 4 coexisting pulmonary and cardiac | Significant p-values (between groups) | |
|---|---|---|---|---|---|---|
| Number of pts, n (%) | 57 (100) | 12 (21) | 35 (61) | 4 (7) | 6 (11) | NA |
| Age at follow-up, y | 25.7 (12.4) | 26.2 (11.9) | 22.5 (11.3) | 35.2 (7.6) | 37.7 (13.5) | 2 vs 4: p = 0.021 |
| Disease duration, y | 17.3 (10.5) | 16.9 (11.5) | 14.7 (9.1) | 28.5 (5.2) | 25.5 (11.7) | 2 vs 3: p = 0.047 |
| Male sex, n (%) | 21 (37) | 2(17) | 11 (31) | 3 (75) | 5 (83) | P = 0.014b |
| Smokers daily disease course; n (%)a | 14 (30) | 2 (18) | 7 (27) | 1 (25) | 4 (67) | NS |
| DAS 1 y | 5.9 (3.9) | 5.9 (4.2) | 4.8 (3.5) | 10.1 (4.1) | 9.0 (3.0) | 2 vs 3: p = 0.039 |
| MDI 1 y | 1.0 (0.0–2.0) | 1.0 (0.0–2.5) | 0.0 (0.0–2.0) | 2.5 (1.5–3.5) | 2.0 (1.0–5.0) | NS |
| Inactive Disease, n (%) | 28 (49) | 6 (50) | 18 (51) | 3 (75) | 1 (17) | NS |
| Calcinosis, n (%) | 21 (37) | 5 (42) | 10 (29) | 2 (50) | 4 (67) | NS |
| Lipodystrophy, n (%) | 10 (18) | 1 (8) | 2 (6) | 2 (50) | 5 (83) | P < 0.001 b |
| Hypertension, disease course, n (%) | 7 (12) | 1(8) | 2 (6) | 2 (50) | 2 (33) | P = 0.025 b |
| TC:HDL ratio N = 50 | 3.9 (2.0) | 3.1 (0.4) | 3.5 (1.3) | 7.4 (3.2) | 5.5 (2.8) |
1 vs 3: p < 0.001 1 vs 4: p = 0.044 2 vs 3: p < 0.001 2 vs 4: p = 0.049 |
| HRV, cSDNN, N = 55 | 39.7 (16.7) | 50.5 (21.8) | 36.8 (13.6) | 42.7 (2.3) | 29.5 (17.6) | 1 vs 4: p = 0.050 |
| NCD, cap/mm | 6.4 (2.1) | 7.1 (1.8) | 6.1 (2.3) | 7.5 (0.6) | 6.4 (2.1) | NS |
| Pred/DMARD, n (%) | 17 (30) | 4 (33) | 10 (29) | 1 (25) | 2 (33) | NS |
| Cum Prednisolone during disease course, g | 7.9 (3.6–12.6) | 8.9 (7.6–11.3) | 4.8 (2.5–10.6) | 17.9 (12.6–26.6) | 14.4 (7.9–27.3) |
2 vs 3; p = 0.008 2 vs 4; p = 0.005 |
| DAS | 4.7 (3.0) | 4.0 (2.4) | 4.5 (2.8) | 3.4 (2.5) | 8.5 (2.8) |
1 vs 4: p = 0.008 2 vs 4: p = 0.008 3 vs 4: p = 0.025 |
| MDI | 4.3 (3.1) | 4.3 (2.2) | 3.5 (2.8) | 5.2 (3.3) | 8.2 (3.8) |
1 vs 4: p = 0.036 2 vs 4: p = 0.003 |
| MMT-8 | 76.5 (4.7) | 77.2 (3.1) | 76.7 (4.1) | 80.0 (0.0) | 71.3 (8.5) |
1 vs 4: p = 0.048 2 vs 4: p = 0.040 3 vs 4: p = 0.019 |
| CMAS | 48.3 (5.4) | 48.8 (3.2) | 48.8 (4.7) | 51.0 (1.4) | 42.8 (10.5) | NS |
| SF-36, PCS a | 50.8 (9.0) | 51.0 (9.7) | 52.3 (7.3) | 56.0 (3.4) | 40.2 (10.9) |
2 vs 4: p = 0.011 3 vs 4: p = 0.023 |
| SF-36, MCSa | 53.4 (7.7) | 51.8 (6.4) | 53.6 (8.1) | 52.7 (5.7) | 55.4 (10.1) | NS |
Variables are assessed at follow-up if not otherwise stated; values are mean (SD) or median (25th—75th percentile) if not otherwise stated; NA not assessed, NS non-significant, DAS Disease activity score, MDI myositis damage index, FU follow-up, TC Total cholesterol, HDL high-density lipoprotein, NCD nailfold capillary density, HRV heart rate variability, NCD Nail fold capillary density, DMARD disease modifying antirheumatic drugs, CMAS child myositis assessment scale, SF-36 Short Form-36, PCS physical assessment scale, MCS mental component scale, Post-hoc tests not run for categorical variables due to low n in several groups. aassessed in patients ≥ 14y at follow-up, n = 47; bpost hoc tests not assessed