Ferdowsi et al reported the Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI). This can help to quantify organ damage in systemic sclerosis. The authors are from the multiple scleroderma research groups.
Patient selection: systemic sclerosis
Parameters:
(1) joint contracture in any small joint of the fingers
(2) joint contracture in a large joint (elbows, knees, others)
(3) sicca symptoms with dry eyes and/or dry mouth
(4) proximal muscle weakness
(5) calcinosis complicated by infection or requiring surgery
(6) digital ulceration
(7) digital amputation required
(8) esophageal dysmotility
(9) esophageal stricture
(10) gastro-esophageal reflux refractory to therapy
(11) gastric antral vascular ectasia
(12) intestinal pseudo-obstruction with dilated small and/or large bowels
(13) body mass index in kilograms per square meter
(14) interstitial lung disease
(15) forced vital capacity as percent of predicted
(16) dependence on home oxygen
(17) pulmonary arterial hypertension
(18) right ventricular dysfunction
(19) myocardial disease
(20) pericardial effusion
(21) history of scleroderma renal crisis
(22) history of scleroderma related kidney disease and eGFR
(23) need for renal replacement therapy
Groupings:
(1) musculoskeletal and skin: 1, 2, 3, 4, 5
(2) vascular: 6, 7
(3) gastrointestinal: 8, 9, 10, 11, 12, 13
(4) respiratory: 14, 15 16
(5) cardiovascular: 17, 18, 19, 20
(6) renal: 21, 22, 23
|
Parameter |
Finding |
Points |
|
joint contracture in any small joint of the fingers |
absent |
0 |
|
|
present |
2 |
|
joint contracture in a large joint (elbows, knees, others |
absent |
0 |
|
|
present |
2 |
|
sicca symptoms with dry eyes and/or dry mouth |
absent |
0 |
|
|
present |
3 |
|
proximal muscle weakness |
absent |
0 |
|
|
present |
3 |
|
calcinosis with complications |
absent |
0 |
|
|
present |
4 |
|
digital ulceration |
absent |
0 |
|
|
present |
2 |
|
digital amputation |
absent |
0 |
|
|
present |
1 |
|
esophageal dysmotility |
absent |
0 |
|
|
present |
1 |
|
esophageal stricture |
absent |
0 |
|
|
present |
1 |
|
GERD refractory to therapy |
absent |
0 |
|
|
present |
1 |
|
gastric antral vascular ectasia |
absent |
0 |
|
|
present |
2 |
|
intestinal pseudo-obstruction |
absent |
0 |
|
|
present |
3 |
|
body mass index |
>= 18.5 kg per sq m |
0 |
|
|
< 18.5 kg per sq m |
2 |
|
interstitial lung disease |
absent or mild |
0 |
|
|
moderate or severe |
2 |
|
FVC as percent of predicted |
>= 70% |
0 |
|
|
< 70% |
4 |
|
dependence on home oxygen |
absent |
0 |
|
|
present |
5 |
|
pulmonary arterial hypertension |
absent |
0 |
|
|
present |
2 |
|
right ventricular dysfunction |
absent or mild |
0 |
|
|
moderate or severe |
5 |
|
myocardial disease |
absent |
0 |
|
|
present |
3 |
|
pericardial effusion |
absent |
0 |
|
|
present |
1 |
|
history of scleroderma renal crisis |
absent |
0 |
|
|
present |
3 |
|
eGFR |
>= 45 mL/kg/1.73 sq m |
0 |
|
|
< 45 mL/kg/1.73 sq m |
1 |
|
need for renal replacement therapy |
absent |
0 |
|
|
present |
2 |
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: 55
• The higher the score the more severe the scleroderma.
|
Score |
Damage |
|
<= 5 |
low |
|
6 to 12 |
moderate |
|
>= 13 |
high |
Performance:
• The area under the ROC curve for mortality is 0.77 in one cohort and 0.73 in another.
Specialty: Immunology/Rheumatology
