Description

Valentini et al developed criteria for disease activity in a patient with diffuse and localized forms of systemic sclerosis. The authors are from multiple hospitals and universities in Europe.


Parameters:

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

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

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

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

(5) change in articular and muscular manifestations (delta articular/muscular - Did your articular or muscular manifestations change during the last month?)

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

(7) Westergren erythrocyte sedimentation rate (ESR)

(8) serum complement (hypocomplementemia)

 

Parameter

Finding

Points

change in skin

improved

0

 

unchanged

0

 

worsened

3

change in vascular manifestations

improved

0

 

unchanged

0

 

worsened

2

change in articular and muscular manifestations

improved

0

 

unchanged

0

 

worsened

1

change in heart and lung

improved

0

 

unchanged

0

 

worsened

4

 

index for diffuse scleroderma =

= SUM(points for all 4 parameters)

 

Parameter

Finding

Points

scleredema

absent

0

 

present

0.5

change in skin

improved

0

 

unchanged

0

 

worsened

2.5

change in vascular manifestations

improved

0

 

unchanged

0

 

worsened

1

arthritis

absent

0

 

present

1

change in heart and lung

improved

0

 

unchanged

0

 

worsened

1.5

ESR by Westergren method

<= 30 mm in first hour

0

 

> 30 mm in first hour

2.5

serum complement

C3 and C4 levels normal

0

 

C3 OR C4 levels decreased

1

 

index for localized scleroderma =

= SUM(points for all 7 parameters)

 

Interpretation:

• minimum index: 0

• maximum for each index: 10

• An index >= 3 indicates active disease.


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