Rubio-Rivas et al reported a score for predicting mortality for a patient with scleroderma. This can help to identify a patient who may require more aggressive management. The authors are from multiple institutions in Spain participating in the RESCLE study and GEAS Study Group.
Patient selection: scleroderma in Spanish adults
Outcome: 15-year all-cause mortality
Parameters:
(1) age at diagnosis
(2) sex
(3) cutaneous subset
(4) mode of onset
(5) interstitial lung disease (ILD)
(6) pulmonary arterial hypertension (PAH)
(7) scleroderma renal crisis (SRC)
(8) heart involvement
(9) cancer
Parameter |
Finding |
Points |
age at diagnosis |
< 55 years |
0 |
|
55 to 65 years |
1 |
|
> 65 years |
2 |
sex |
female |
0 |
|
male |
1 |
cutaneous subset |
lcSSC (limited cutaneous) |
-1 |
|
other |
0 |
mode of onset |
Raynaud phenomenon (RP) |
0 |
|
other than RP |
1 |
ILD |
absent |
0 |
|
present |
1 |
PAH |
absent |
0 |
|
present |
1 |
SRC |
absent |
0 |
|
present |
2 |
heart involvement |
absent |
0 |
|
present |
1 |
cancer |
absent |
0 |
|
present |
1 |
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: -1
• maximum score: 10
Score |
Risk Group |
Mortality at 15 Years |
-1 or 1 |
low |
5-7% |
2 or 3 |
intermediate |
26-27% |
4 to 10 |
high |
48-59% |
Performance:
• The area under the ROC curve is 0.80.
Specialty: Immunology/Rheumatology