Article Figures & Data
Figures
- Figure 1.
HRCT abnormalities in JMCTD. A. ILD extent in percent of total lung parenchyma. B. HRCT image of a typical patient with JMCTD, with a fine reticular pattern grade 1 and 2, and 7% of total lung volume involved. HRCT: high-resolution computed tomography; JMCTD: juvenile mixed connective tissue disease; ILD: interstitial lung disease.
Tables
- Table 1.
Characteristics in patients with JMCTD and controls from the general population. Values are mean (SD) or median (range) unless otherwise specified.
Variables Patients with JMCTD, n = 52 Controls, n = 52 p Age at onset, yrs 11.7 (3.1) NA Age at diagnosis, yrs 14.4 (4.4) NA Disease duration, from onset 16.2 (10.3) NA Female sex, n (%) 44 (85) 44 (85) 1.00 BMI, kg/m2 22.7 (3.5) 23.4 (3.0) 0.239 Height, cm 166.0 (7.5) 170.1 (8.5) 0.016 Weight, kg 63.0 (12.1) 68.1 (12.1) 0.033 Current smokers, n (%) 7 (14) 9 (17) 0.586 Never smokers, n (%) 35 (67) 34 (65) 0.832 Hemoglobin, g/100 ml 13.5 (1.2) 13.6 (1.1) 0.809 Vigorous physical activity, h/week 2.0 (0–25) 2.0 (0–55) 0.400 Moderate physical activity, h/week 2.0 (0–28) 1.2 (0–30.0) 0.278 JMCTD: juvenile mixed connective tissue disease; NA: not assessed; BMI: body mass index.
- Table 2.
Pulmonary function after mean 16.2 years of disease duration in patients with JMCTD and controls. Values are mean (SD) or median (range) unless otherwise specified.
Variables Patients with JMCTD, n = 52 Controls, n = 52 Mean Difference, % p FVC, l 3.4 (0.6) 4.3 (1.0) < 0.001 FVC% 89 (14.7) 109 (11.9) 20 < 0.001 FEV1, l 2.9 (0.5) 3.5 (0.8) < 0.001 FEV1% 89 (13.7) 103 (11.0) 14 < 0.001 FEV1/FVC ratio 0.86 (0.06) 0.82 (0.07) 0.001 DLCO mmol/kPa 6.8 (1.3) 9.2 (2.4) < 0.001 DLCO% 73 (12.5) 94 (16.0) 21 < 0.001 DLCO/VA 1.6 (0.2) 1.8 (1.0) 0.282 DLCO/VA% 88 (12.7) 92 (13.4) 4 0.207 TLC, l 4.8 (1.0) 6.0 (1.2) < 0.001 TLC% 90 (13.1) 108 (10.6) 18 < 0.001 Abnormal PFT* 36 (69) 13 (25) < 0.001 FVC < 80%, n (%) 11 (21) 0 < 0.001 FEV1 < 80%, n (%) 12 (23) 2 (4) < 0.001 FEV1/FVC < 0.70, n (%) 1 (2) 3 (6) 0.617 DLCO < 80%, n (%) 35 (67) 9 (17) < 0.001 DLCO < 70%, n (%) 19 (37) 1 (2) < 0.001 DLCO/VA < 80%, n (%) 12 (23) 8 (15) 0.338 TLC < 80%, n (%) 6 (12) 0 0.009 6MWT, m 634.3 (76.5) 698.9 (86.6) 0.002 Borg CR10 scale 2.0 (0–4.0) 1.0 (0–3.0) 0.072 ↵* FVC, FEV1, and/or DLCO < 80% of expected value, and/or FVC/FEV1 < 70% of predicted. JMCTD: juvenile mixed connective tissue disease; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; VC: vital capacity; VA: alveolar volume; TLC: total lung capacity; PFT: pulmonary function tests; 6MWT: 6-min walk test; CR: category ratio.
- Table 3.
HRCT findings in 52 patients with JMCTD after mean 16.2 years of disease duration.
Variables n (%) ILD on HRCT* 14 (27) Ground-glass attenuation 2 (4) Reticular pattern present 13 (25) Grade 1 12 (23) Grade 2 6 (12) Grade 3 1 (2) Interlobular septal thickening 0 Airspace consolidations 0 Bronchiectasis 1 (2) ↵* Defined as reticular pattern and/or ground-glass attenuation, interlobular septal thickening, airspace consolidations, and bronchiectasis. HRCT: high-resolution computed tomography; JMCTD: juvenile mixed connective tissue disease; ILD: interstitial lung disease.
- Table 4.
Patient and disease characteristics in 52 patients with JMCTD with and without ILD at examination after 16.2 years disease duration. Values are n (%) or median (range) unless otherwise specified.
Characteristics No ILD, n = 38 ILD, n = 14 p At examination Female sex 34 (90) 10 (71) NS Age, yrs, mean (SD) 27.1 (10.3) 30.4 (10.2) NS Disease duration, from symptom onset 15.1 (10.2) 19.4 (10.2) NS BMI, kg/m2, mean (SD) 23.3 (3.0) 23.7 (2.9) NS Current smokers 3 (8) 4 (29) NS Never smokers 26 (68) 9 (64) NS SLE-like disease 22 (58) 5 (36) NS SSc-like disease 22 (58) 14 (100) 0.025 PM-like disease 2 (5) 0 NS PFT Impaired PFT^ 27 (73) 9 (64) NS DLCO < 80% 26 (70) 9 (64) NS DLCO < 70% 13 (34) 6 (43) NS FVC < 80% 5 (14) 6 (43) 0.023 TLC < 80% 1 (3) 5 (36) 0.002 FEV1/FVC < 0.70 1 (3) 0 NS 6MWT, m, mean (SD) 626.5 (77.1) 626.9 (79.8) NS Disease activity at examination Active disease 26 (68) 9 (64) NS No. active joints 0 (0–11) 0 (0–2) NS Anti-RNP, titer × 103, U/l 83.5 (0–240) 234.0 (17–240) NS ESR, mm/h 9.0 (2–54) 8.0 (2–30) NS CRP, mg/l 0.7 (0–15) 0.7 (0–9.3) NS Anti-Ro52-positive 1 (3) 0 NS PGA, 10 cm VAS 1.5 (0–58.0) 2.9 (0.2–5.7) NS Rodnan skin score 0 (0–4) 0 (0–11) NS ↵^ FVC, FEV1, and/or DLCO < 80% of predicted, and/or FEV1/FVC < 0.7. JMCTD: juvenile mixed connective tissue disease; ILD: interstitial lung disease; BMI: body mass index; SLE: systemic lupus erythematosus; SSc: systemic sclerosis; PM: polymyositis; PFT: pulmonary function test; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; TLC: total lung capacity; 6MWT: 6-min walk test; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; PGA: physician’s global assessment; VAS: visual analog scale; NS: not significant.
- Table 5.
Progression of HRCT findings (n = 37) and changes in PFT (n = 38) in patients with JMCTD. Values are mean (SD) or median (range) unless otherwise specified.
Variables Time 1 Time 2 p Disease duration at time of HRCT, yrs 8.8 (0.6–33.6) 17.8 (3.1–40.2) NA Disease duration at time of PFT, yrs 11.6 (3.3–30.9) 17.7 (2.3–41.2) NA Age at PFT examination, yrs 21.9 (6.5) 32.2 (6.7) NA Age at HRCT, yrs 21.7 (7.3) 29.3 (8.6) NA Time between HRCT scans 8.3 (2.7) NA Time between PFT 10.3 (1.0) NA FVC% 92.7 (11.4) 85.6 (15.6) 0.004 FEV1% 94.6 (12.0) 85.9 (10.3) 0.001 FEV1/FVC ratio 0.87 (0.05) 0.84 (0.05) NS DLCO% 72.2 (14.0) 70.5 (11.3) NS DLCO/VA% 88.2 (15.7) 88.2 (15.1) NS ILD present, n (%) 9 (24) 10 (27) NS Ground-glass attenuation, n (%) 3 (8) 0 NS Reticular pattern, n (%) 7 (19) 10 (27) NS Grade 1 8 (22) 9 (24) NS Grade 2 4 (11) 6 (16) NS Grade 3 0 1 (3) NS Lung parenchyma with ILD, % 2.0 (1–18) 4.0 (1–18) NS HRCT: high-resolution computed tomography; PFT: pulmonary function tests; JMCTD: juvenile mixed connective tissue disease; ILD: interstitial lung disease; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusion capacity of carbon monoxide; VA: alveolar volume; NA: not assessed; NS: not significant.
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