From the Guidelines
The management of scleroderma should be guided by the latest EULAR recommendations, which emphasize a multidisciplinary approach tailored to the specific manifestations of the disease, including the use of novel therapies such as mycophenolate mofetil, nintedanib, rituximab, and tocilizumab for skin fibrosis and interstitial lung disease. The treatment of scleroderma is complex and requires a comprehensive approach to manage its various manifestations, including skin thickening, Raynaud's phenomenon, gastrointestinal symptoms, pulmonary arterial hypertension, and interstitial lung disease.
Key Considerations
- For skin thickening, mycophenolate mofetil (1-2 g daily) or novel therapies such as rituximab and tocilizumab may be used 1.
- Raynaud's phenomenon is managed with calcium channel blockers like nifedipine (30-60 mg daily) or amlodipine (5-10 mg daily), and in severe cases, phosphodiesterase-5 inhibitors such as sildenafil (20 mg three times daily) may be added 1.
- Gastrointestinal symptoms require proton pump inhibitors like omeprazole (20-40 mg daily) for reflux, and prokinetic agents such as metoclopramide (10 mg before meals) for motility issues.
- Pulmonary arterial hypertension may be treated with endothelin receptor antagonists (bosentan 62.5-125 mg twice daily), while interstitial lung disease often requires cyclophosphamide or mycophenolate mofetil, and in some cases, anti-fibrotic therapy such as nintedanib may be added 1.
Non-Pharmacological Approaches
- Non-pharmacological approaches include physical therapy to maintain joint mobility, occupational therapy for daily activities, and protection from cold exposure.
- Regular monitoring of organ function is essential to detect and address complications early.
- Patient education about the disease course and self-management strategies is also crucial for optimal outcomes. The latest EULAR recommendations provide a framework for the management of scleroderma, emphasizing the importance of a multidisciplinary approach and the use of novel therapies to improve outcomes for patients with this complex disease 1.
From the Research
Management Options for Scleroderma
The management of scleroderma involves a range of treatment options, including:
- Vasodilators, such as calcium channel blockers and angiotensin-converting enzyme inhibitors, to improve peripheral blood circulation 2
- Immunosuppressant drugs, such as methotrexate and cyclophosphamide, to prevent the synthesis and release of harmful cytokines 2
- Antifibrotic agents, such as D-penicillamine and colchicine, to inhibit or reduce fibrosis 2
- Endothelin receptor antagonists and direct renin inhibitors for the treatment of scleroderma renal crisis (SRC) 3
- Biologic agents targeting collagen, cytokines, and cell surface molecules, which may have promising therapeutic effects in systemic sclerosis (SSc) 4
Treatment of Specific Complications
For specific complications of scleroderma, the following treatment options are recommended:
- Scleroderma renal crisis (SRC): angiotensin-converting enzyme inhibitors as first-line therapy, with the addition of calcium channel blockers, angiotensin receptor blockers, or alpha-blockers as second-line therapy 5
- Pulmonary arterial hypertension (PAH): endothelin receptor agonists as first-line therapy, with the addition of phosphodiesterase-5 inhibitors and prostanoids as second-line therapy 5
- Raynaud's phenomenon: calcium channel blockers as first-line therapy, with the addition of phosphodiesterase-5 inhibitors, angiotensin receptor blockers, and prostanoids as second-line therapy 5
- Digital ulcers: calcium channel blockers as first-line therapy, with the addition of phosphodiesterase-5 inhibitors, endothelin receptor agonists, and prostanoids as second-line therapy 5
- Interstitial lung disease/pulmonary fibrosis: intravenous cyclophosphamide and mycophenolate mofetil as induction therapy, with mycophenolate mofetil as maintenance therapy 5
Novel Investigational Agents
Several novel investigational agents are being studied for the treatment of scleroderma, including:
- Rituximab, tocilizumab, and IVIG for the treatment of active SSc skin or lung disease 6
- Bortezomib, LPA-1 antagonists, anti-CCN2 therapy, anti-IL-13, and thrombin antagonists for the treatment of fibrosis 6
- Agents targeting intracellular molecular pathways and matricellular proteins, which may be a novel area of investigation 6