From the Guidelines
Treatment of Systemic Sclerosis (SSc) should be individualized and based on the specific organs involved, with a focus on managing symptoms and preventing complications, as recommended by the 2023 EULAR update 1. The treatment options for SSc are diverse and depend on the organ systems affected. For Raynaud's phenomenon, dihydropyridine-type calcium antagonists, such as nifedipine, should be used as first-line therapy, with PDE5 inhibitors and intravenous iloprost considered for severe cases or those who fail oral therapy 1. For digital ulcers, PDE5 inhibitors and/or intravenous iloprost should be considered, with bosentan as an option for reducing the number of new digital ulcers 1. In patients with SSc-associated pulmonary arterial hypertension (SSc-PAH), a combination of PDE5 inhibitors and endothelin receptor antagonists should be considered as first-line treatment, with intravenous epoprostenol or other prostacyclin analogues reserved for advanced disease 1. For interstitial lung disease (ILD), mycophenolate mofetil, cyclophosphamide, or rituximab should be considered, with nintedanib as an option for patients with progressive fibrosis 1. Skin thickening can be treated with methotrexate, mycophenolate mofetil, or rituximab, with tocilizumab considered for early, inflammatory disease 1. Regular monitoring by a rheumatologist and a multidisciplinary approach involving other specialists are essential for optimal management of SSc, as highlighted in recent reviews 2, 3. Lifestyle modifications, including protection from cold, smoking cessation, and regular exercise, are also important components of managing this chronic autoimmune condition.
From the Research
Treatment Options for Systemic Sclerosis (SSc)
The treatment of Systemic Sclerosis (SSc) is challenging and involves various therapeutic approaches. The following are some of the treatment options for SSc:
- Vasodilators: + Calcium channel blockers (e.g., nifedipine) 4, 5, 6, 7 + Angiotensin-converting enzyme inhibitors (e.g., captopril, losartan) 4, 5, 6, 7 + Prostacyclin analogues (e.g., iloprost, epoprostenol) 4, 5, 6, 7
- Immunosuppressant drugs: + Methotrexate 4, 5, 8, 6, 7 + Cyclophosphamide 4, 5, 8, 6, 7 + Cyclosporine 4, 5 + Mycophenolate mofetil 8, 6
- Antifibrotic agents: + D-penicillamine 4, 5, 8 + Colchicine 4 + Interferon gamma 4 + Relaxin 4, 5, 8
- Endothelin receptor antagonists: + Bosentan 5, 8, 6, 7
- Phosphodiesterase-5 inhibitors: + Sildenafil 8, 6, 7
- Other treatment options: + Extracorporeal photopheresis 4, 8 + Stem cell transplantation 4, 5, 8, 6 + Lung transplantation 4, 5 + Autologous stem cell transplantation 4 + Etanercept 4 + Thalidomide 4 + Minocycline 4 + Psoralen-UV-A 4 + IVIg 5, 8 + N-acetylcysteine 5 + Anti-TGF-beta antibodies 8 + Tyrosine kinase inhibitors 8 + Rituximab 8 + Fluoxetine 8, 7 + Pirfenidone 8 + Halofuginone 8 + Collagen tolerance induction 8 + Corticosteroids 6, 7 + Hydroxychloroquine 6 + Proton pump inhibitors 6, 7 + Metoclopramide 7 + Erythromycin 7 + Octreotide 7 + Oral antibiotics 7 + Sitaxentan 7 + Ambrisentan 7 + Treprostinil 7