Evaluation of Skin Fibrosis in Systemic Sclerosis

Method of Evaluation

The examiner palpates the skin at 17 specific anatomical sites and assigns a score of 0 to 3 for each site, with a total score ranging from 0 to 51, as recommended by the European League Against Rheumatism 1, 2
The 17 anatomical sites evaluated include fingers, hands, forearms, arms, chest, abdomen, thighs, legs, and feet, with a score of 0 indicating normal skin, 1 indicating slight thickening, 2 indicating moderate thickening, and 3 indicating severe thickening, as defined by the American College of Rheumatology 1
The total score is calculated by summing all individual scores from the 17 sites, giving a maximum possible score of 51, which is a reliable measure of skin fibrosis severity, according to the European League Against Rheumatism 1, 2

The minimal clinically important difference of the modified Rodnan skin score (mRSS) has been estimated to be between 3.5 and 5.3 points, indicating a significant change in skin fibrosis, as reported by the American College of Rheumatology 1
In diffuse cutaneous systemic sclerosis (dcSSc), the mRSS typically increases over the first 4 years of disease, then somewhat regresses, although many patients do not follow this pattern, according to the European League Against Rheumatism 1, 2
A high mRSS (>24) at baseline is associated with an increased risk of mortality and lower rates of survival without progression, suggesting that patients should be evaluated earlier in the disease course, as recommended by the American College of Rheumatology 3

The mRSS is the most commonly used tool to assess the extent of skin fibrosis in systemic sclerosis, and is a reliable measure of disease severity, according to the European League Against Rheumatism 1, 2
High skin scores or moderate scores with progressive interstitial lung disease are criteria for considering autologous hematopoietic stem cell transplantation in patients with early dcSSc, as recommended by the American College of Rheumatology 4, 5

A high mRSS is a risk factor for the development of interstitial lung disease in systemic sclerosis, indicating a need for close monitoring and early intervention, as reported by the European Respiratory Society 6, 7
Rapid skin progression (rapid increase in mRSS) is a risk factor for scleroderma renal crisis, highlighting the importance of regular assessments and timely treatment, according to the American College of Rheumatology 1