Description

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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