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.
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