Table 2 Frequency of Scleroderma Clinical Trials Consortium Damage Index items for juvenile systemic sclerosis patients in the Peking Union Medical College Hospital cohort
Items | All patients(n = 64) | Patients with follow-up (n = 52) | |
|---|---|---|---|
Baseline | Baseline (n = 52) | Follow-up (n = 52) | |
Musculoskeletal and skin | |||
Joint contracture (small joints) | 26(40.6) | 22(42.3) | 28(53.8) |
Joint contracture (large joints) | 14(21.9) | 11(21.2) | 16(30.8) |
Sicca symptoms | 5(7.8) | 3(5.8) | 5(9.6) |
Proximal muscle weakness | 7(10.9) | 5(9.6) | 1(1.9) |
Calcinosis complicated by infection or requiring surgery | 2(3.1) | 4(7.7) | 4(7.7) |
Vascular | |||
Digital ulceration | 22(34.4) | 18(34.6) | 24(46.2) |
Digital amputation required | 4(6.3) | 3(5.8) | 3(5.8) |
Gastrointestinal | |||
Esophageal dysmotility | 4(6.3) | 4(7.7) | 4(7.7) |
Esophageal stricture | 0 | 0 | 1(1.9) |
Refractory gastro-esophageal reflux disease | 3(4.7) | 3(5.8) | 4(7.7) |
GAVE | 0 | 0 | 0 |
Pseudo-obstruction | 1(1.6) | 0 | 1(1.9) |
BMI < 3rd percentile for children of same-age and sex or weight loss of > 10% in past 12 months | 4(6.3) | 2(3.8) | 2(3.8) |
Respiratory | |||
ILD > 20% extent on HRCT | 23(35.9) | 19(36.5) | 25(48.1) |
FVC < 70% | 10(15.6) | 8(15.4) | 13(25.0) |
Dependence on home oxygen | 1(1.6) | 0 | 4(7.7) |
Cardiovascular | |||
PAH | 4(6.3) | 4(7.7) | 6(11.5) |
Moderate to severe right ventricular dysfunction | 2(3.1) | 2(3.8) | 2(3.8) |
Myocardial disease | 2(3.1) | 2(3.8) | 3(5.8) |
Moderate to large pericardial effusion | 0 | 0 | 0 |
Renal | |||
History of SRC | 0 | 0 | 0 |
eGFR < 45 mL/min/1.73 m2 | 0 | 0 | 0 |
CKD stage 5 and need for renal replacement therapy | 0 | 0 | 0 |
SCTC-DI (score) | 4.0 (2.0, 7.0) | 4.0 (2.0, 7.8) | 6.0(2.0, 9.0) |