From the Guidelines
The initial treatment approach for patients with SCL-70 positive skin manifestations of systemic sclerosis typically involves immunosuppressive therapy with mycophenolate mofetil (MMF) at doses of 1-3g daily in divided doses, as recommended by the most recent EULAR guidelines 1. This approach is based on the latest evidence from the 2023 EULAR update, which suggests that MMF, methotrexate, and/or rituximab should be considered for the treatment of SSc skin fibrosis, with a level of evidence of 1a-b and a strength of recommendation of A/B 1. The choice of treatment may depend on the severity of skin involvement and the presence of other organ involvement, such as interstitial lung disease. For patients with rapidly progressive diffuse cutaneous systemic sclerosis, methotrexate (15-25mg weekly) may be used as an alternative or in combination with MMF, as supported by studies such as the Scleroderma Lung Study I and II 1. Supportive care is equally important, including moisturizers for dry skin, calcium channel blockers like nifedipine (30-60mg daily) for Raynaud's phenomenon, and proton pump inhibitors for reflux symptoms. Physical therapy should be initiated early to maintain joint mobility and hand function. Regular monitoring for organ involvement is essential, as SCL-70 positivity is associated with a higher risk of interstitial lung disease and other complications, as highlighted in the state-of-the-art evidence in the treatment of systemic sclerosis 1. Some key points to consider in the treatment of SSc skin manifestations include:
- The use of MMF as a first-line treatment for skin fibrosis, with a level of agreement of 7.6 (3.2) and a percentage of level of agreement greater than 8 of 72% 1
- The potential benefits of methotrexate as an alternative or adjunctive treatment, with a between-group difference in mRSS of approximately 5 points in favor of methotrexate, compared with placebo 1
- The importance of supportive care and regular monitoring for organ involvement, as SCL-70 positivity is associated with a higher risk of complications 1
From the Research
Initial Treatment Approach for SCL-70 Positive Skin Manifestations
The initial treatment approach for patients with SCL-70 (anti-topoisomerase I antibody) positive skin manifestations of systemic sclerosis involves immunosuppressive therapies.
- The treatment focuses on halting the progression of skin involvement and internal organ involvement, such as interstitial lung disease 2.
- Immunosuppressants, including methotrexate, mycophenolate mofetil, and cyclophosphamide, are commonly used to treat progressing skin involvement and interstitial lung disease in systemic sclerosis 2.
- Mycophenolate mofetil (MMF) has been shown to halt the progression of rapidly progressive diffuse cutaneous systemic sclerosis (rp-dcSSc) cutaneous and pulmonary involvement 3.
- The use of immunosuppressants, particularly MMF, has been associated with improved outcomes in patients with SCL-70 positive skin manifestations, including reduced skin thickening and improved lung function 2, 3.
- In some cases, anti-fibrotic agents like nintedanib may be considered for patients with progressive lung fibrosis 4.
- The presence of anti-Scl-70 antibodies is highly specific for systemic sclerosis and is often associated with diffuse cutaneous involvement and internal organ involvement 5, 6.