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.
Purpose: To evaluate a patient with diffuse or localized systemic sclerosis using the criteria of Valentini and the European Scleroderma Study Group.
Specialty: Immunology/Rheumatology
Objective: criteria for diagnosis
ICD-10: M34,