Criteria of Valentini et al (European Scleroderma Study Group) for Activity of Systemic Sclerosis

Parameters:

(1) modified Rodnan skin score (17 sites scored 0 to 3, maximum 51)

(2) scleredema (increase in soft tissue mass due to dermal imbibition, with abolishment of skin contours and skin folds)

(3) change in skin (delta skin - Did your skin manifestations change during the last month?)

(4) digital necrosis (active digital ulcers ranging from small infarcts on the tips of fingers to gangrene of whole digits)

(5) change in vascular manifestations (delta vascular - Did your vascular manifestations change during the last month?)

(6) arthritis (symmetrical swelling and tenderness of the peripheral joints)

(7) carbon monoxide diffuse capacity (DLCO) by the single breath method

(8) change in heart and lung (delta heart/lung - Did your cardiopulmonary manifestations change during the last month?)

(9) Westergren erythrocyte sedimentation rate (ESR)

(10) serum complement (hypocomplementemia)

where:

• Earlier versions of the criteria used the Kahaleh total skin score, with a cutoff > 20.

total index =

= SUM(points for all 10 parameters)

Interpretation:

• minimum index: 0

• maximum index: 10

• An index >= 3 indicates active disease.