From the Guidelines
Treatment for scleroderma should be individualized and focused on managing symptoms, preventing complications, and improving quality of life, with the most recent EULAR recommendations suggesting the use of mycophenolate mofetil, nintedanib, rituximab, and tocilizumab for key fibrotic manifestations. The approach typically includes medications to control inflammation and immune system activity, such as corticosteroids (prednisone), immunosuppressants (methotrexate, mycophenolate mofetil, cyclophosphamide), and biologics (rituximab) 1. For skin thickening, medications like methotrexate (starting at 15mg weekly) or mycophenolate mofetil (1-2g daily) may be used, with mycophenolate mofetil and rituximab being recommended for the treatment of skin fibrosis 1. Raynaud's phenomenon is treated with calcium channel blockers like nifedipine (30-60mg daily) or amlodipine (5-10mg daily), with dihydropyridine-type calcium antagonists being recommended as first-line therapy 1. Gastrointestinal issues often require proton pump inhibitors such as omeprazole (20-40mg daily) for acid reflux, with proton pump inhibitors being recommended for the treatment of SSc-GERD 1. Pulmonary arterial hypertension may be managed with medications like sildenafil, bosentan, or ambrisentan, with a combination of PDE5 inhibitors and endothelin receptor antagonists being recommended as first-line treatment 1. Physical therapy is crucial to maintain joint mobility and muscle strength. Patients should protect their hands from cold, moisturize skin regularly, elevate the head of the bed for reflux symptoms, and perform regular stretching exercises.
Some key points to consider in the treatment of scleroderma include:
- Autologous haematopoietic stem cell transplantation can improve survival in patients with early diffuse cutaneous SSc 1
- Immunosuppressives and some biologic agents can soften skin and change the natural history of early diffuse cutaneous SSc 1
- Appropriate treatment for patients with early limited cutaneous SSc is unknown, and further research is needed 1
- Interstitial lung disease is usually treated with mycophenolate mofetil as the initial therapy, and then other immunosuppressives or biologic agents 1
Overall, the goal of treatment is to slow disease progression and improve quality of life by targeting the underlying immune dysfunction and fibrosis, with the most recent EULAR recommendations providing guidance on the use of various medications for different manifestations of scleroderma 1.
From the Research
Treatment Options for Scleroderma
The treatment of systemic sclerosis (scleroderma) is challenging and involves various therapeutic approaches. The following are some of the treatment options for scleroderma:
- Vasodilators: calcium channel blockers (nifedipine), angiotensin-converting enzyme inhibitors (captopril, losartan potassium), and prostaglandins (iloprost, epoprostenol) 2
- Immunosuppressant drugs: methotrexate, cyclosporine, cyclophosphamide, and extracorporeal photopheresis 2
- Antifibrotic agents: D-penicillamine, colchicine, interferon gamma, and relaxin 2
- Corticosteroids, vitamin D analogues (calcitriol, calcipotriene), UV-A, and methotrexate for localized scleroderma 2
- Endothelin receptor antagonism and renin inhibition for scleroderma kidney 3
- Angiotensin-converting enzyme inhibitors for scleroderma renal crisis 4, 5
- Mycophenolate mofetil for treatment of diffuse cutaneous systemic sclerosis 6
Specific Treatment Approaches
For scleroderma renal crisis, the first-line therapy is an angiotensin-converting enzyme inhibitor (ACEi) 4, 5. For pulmonary arterial hypertension, endothelin receptor agonists (ERAs) are the first treatment in mild cases, followed by adding a phosphodiesterase-5 inhibitor (PDE5i) and then a prostanoid 4. For Raynaud's phenomenon, calcium channel blockers (CCBs) are the initial treatment, followed by adding a PDE5i or prostanoid 4.
Treatment Outcomes
The use of ACE inhibitors has dramatically improved the survival of patients with scleroderma renal crisis 5. Mycophenolate mofetil (MMF) may benefit skin disease in patients with diffuse scleroderma, with improvement in modified Rodnan skin scores (mRSS) and quality of life measures 6.