From the Guidelines
Scleroderma patients should be treated with mycophenolate mofetil, nintedanib, rituximab, or tocilizumab for skin fibrosis and interstitial lung disease, as recommended by the 2023 EULAR update 1. The treatment of scleroderma, also known as systemic sclerosis, has evolved with the introduction of new therapies and updates to existing guidelines.
Key Considerations
- The 2023 EULAR recommendations provide a comprehensive approach to managing various clinical and organ domains affected by scleroderma, including Raynaud’s phenomenon, digital ulcers, pulmonary arterial hypertension, and skin fibrosis 1.
- Novel recommendations for the use of mycophenolate mofetil, nintedanib, rituximab, and tocilizumab offer new treatment options for skin fibrosis and interstitial lung disease, which are crucial disease manifestations 1.
- First-line and second-line interventions are outlined, providing increased utility for rheumatology practitioners in managing scleroderma patients.
Treatment Approach
- Mycophenolate mofetil is recommended for the treatment of skin fibrosis and interstitial lung disease, with a typical dosage of 1-3g daily 1.
- Nintedanib is also recommended for the treatment of interstitial lung disease, with a typical dosage of 150-300mg twice daily 1.
- Rituximab and tocilizumab may be considered for the treatment of skin fibrosis and interstitial lung disease, with dosages varying depending on the specific clinical context 1.
Monitoring and Follow-up
- Regular monitoring by a rheumatologist is essential to assess disease progression and adjust treatment as needed.
- Early intervention for organ complications significantly improves outcomes in scleroderma patients.
From the Research
Scleroderma Overview
- Scleroderma is a multisystem autoimmune disease characterized by abnormal immune activation, leading to vascular and fibrotic disease manifestations 2.
- It can affect various parts of the body, including the skin, lungs, and internal organs.
Treatment Options
- Nonselective immunosuppression, particularly cyclophosphamide, is a common treatment for progressing skin involvement and active interstitial lung disease 2.
- Mycophenolate mofetil is a promising alternative to cyclophosphamide, with studies showing its efficacy in treating scleroderma-associated interstitial lung disease 3, 4.
- Other treatment options include cyclosporine, sirolimus, alefacept, and rituximab, although their use may be limited by modest efficacy and serious side effects 2.
- Hematopoietic stem cell transplantation can be effective for severe fibrotic skin disease, but toxicity remains a concern 2.
Disease Management
- Induction treatment with cyclophosphamide pulse therapy followed by maintenance treatment with mild immunosuppressive agents can help preserve lung function in scleroderma patients 5.
- Methotrexate is also used to treat scleroderma and mixed connective tissue disease, although its efficacy may vary depending on the individual patient 6.
- Long-term treatment with mycophenolate mofetil can lead to stable pulmonary function and improved clinical outcomes in patients with scleroderma-associated interstitial lung disease 4.