The American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) presented criteria for the diagnosis of systemic sclerosis (SSc) in 2013. The authors are from multiple institutions in the United States, Europe, Canada and New Zealand.
Patient selection: skin thickening involving the fingers not due to a scleroderma-like disorder
Parameters:
(1) skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints
(2) skin thickening of the fingers
(3) fingertip lesions
(4) telangiectasia
(5) abnormal nailfold capillaries
(6) pulmonary arterial hypertension and/or interstitial disease
(7) Raynaud's phenomenon
(8) SSC-related autoantibodies (anticentromere, anti-topoisomerase I [anti-Scl-70], or anti-RNA polymerase III)
If there is skin thickening of both hands extending proximal to the metacarpophalangeal joints, then the patient has systemic sclerosis and is assigned 9 points.
If skin thickening of the fingers of both hands is absent then the other parameters are evaluated.
|
Parameter
|
Finding
|
Points
|
|
skin thickening of the fingers
|
none
|
0
|
|
|
puffy fingers
|
2
|
|
|
sclerodactyly
|
4
|
|
fingertip lesions
|
none
|
0
|
|
|
digital tip ulcers
|
2
|
|
|
fingertip pitting scars
|
3
|
|
telangiectasia
|
absent
|
0
|
|
|
present
|
2
|
|
nailfold capillaries
|
normal
|
0
|
|
|
abnormal
|
2
|
|
lung involvement
|
none
|
0
|
|
|
pulmonary arterial hypertension
|
2
|
|
|
interstitial lung disease
|
2
|
|
|
both
|
2
|
|
Raynaud's phenomenon
|
absent
|
0
|
|
|
present
|
3
|
|
SSc-related autoantibodies
|
absent
|
0
|
|
|
present
|
3
|
where:
• Sclerodactyly of the fingers is distal to the metacarpophalangeal joints but proximal to the proximal to the proximal interphalangeal joint.
• Scoring of autoantibodies is based on text on 2742 which uses "or".
total score =
= SUM(points for all of the parameters scored)
Interpretation:
• minimum score: 0
• A score >=9 indicated definite systemic sclerosis.
• Skin thickening of both hands is sufficient criterion for the diagnosis.
