What are the latest treatments for Systemic Sclerosis (SSc)?

From the Guidelines

The most effective treatment for systemic sclerosis involves a multi-faceted approach, including immunosuppressants like mycophenolate mofetil, and targeted therapies such as nintedanib and rituximab, as recommended by the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline 1. The treatment of systemic sclerosis (SSc) has evolved significantly, with a focus on managing specific symptoms and preventing complications.

• For interstitial lung disease (ILD) associated with SSc, mycophenolate mofetil is the preferred first-line therapy, with tocilizumab and cyclophosphamide as additional options 1.
• In patients with rapidly progressive early diffuse cutaneous SSc, autologous haematopoietic stem cell transplantation may be considered, as it has been shown to improve survival 1.
• Pulmonary arterial hypertension is frequently treated with initial combination therapy, including phosphodiesterase 5 inhibitors and endothelin receptor antagonists, with the addition of a prostacyclin analogue if necessary 1.
• Raynaud's phenomenon and digital ulcers are treated with dihydropyridine calcium channel blockers, such as nifedipine, and phosphodiesterase 5 inhibitors, with bosentan reducing the development of new digital ulcers 1.
• The 2023 EULAR recommendations for the treatment of systemic sclerosis also emphasize the use of mycophenolate mofetil, nintedanib, rituximab, and tocilizumab for the treatment of skin fibrosis and ILD 1. The treatment approach should be individualized based on organ involvement, as SSc affects multiple systems with varying severity across patients.
• Gastrointestinal symptoms are managed with proton pump inhibitors for reflux and prokinetic agents for motility issues.
• Scleroderma renal crisis is treated with ACE inhibitors.
• Autologous hematopoietic stem cell transplantation has shown promise for selected patients with rapidly progressive disease. It is essential to consider the most recent and highest-quality evidence when making treatment decisions for patients with systemic sclerosis, as recommended by the 2023 ACR/CHEST guideline 1 and the 2023 EULAR recommendations 1.

From the FDA Drug Label

Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice Adverse events reported with the use of intravenous iloprost in patients with frostbite from the published literature include headache, flushing, palpitations/tachycardia, nausea, vomiting, dizziness, and hypotension Pre-marketing safety data on AURLUMYN were obtained from 116 patients with Systemic Sclerosis receiving iloprost in 2 multicenter, double-blind, randomized, placebo-controlled studies in patients with Systemic Sclerosis experiencing symptomatic digital ischemic episodes (Raynaud's Phenomenon)

Last treatments for systemic sclerosis are not explicitly mentioned in the provided text. However, iloprost (IV) is used to treat symptomatic digital ischemic episodes (Raynaud's Phenomenon) in patients with Systemic Sclerosis. The treatment involves a continuous infusion of iloprost over 6 hours each day for 5 consecutive days, with the dose adjusted according to individual tolerability within the range of 0.5 to 2.0 ng/kg/min 2.

From the Research

Current Treatments for Systemic Sclerosis

• The treatment of systemic sclerosis (SSc) is complex and involves various therapeutic approaches, including immunosuppressive agents, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors 3.
• Cyclophosphamide (CYC) has been a gold standard of treatment for severe progressive SSc, but its use is time-limited due to significant adverse events, and other immunosuppressive and biological agents have shown efficacy with better safety profiles 4.
• Autologous hematopoietic stem cell transplantation (HSCT) has emerged as a potential intervention for severe SSc refractory to conventional treatment, with non-myeloablative selective HSCT and myeloablative selective HSCT showing improvement in event-free survival and skin thickness compared to cyclophosphamide 5.

Novel Therapeutic Strategies

• Novel therapeutic strategies and new medications under development for the treatment of SSc include combining therapies to address individual manifestations of the disease, controlling the complex biological network, progressive vasculopathy, and fibrosis, as well as manifestations of end-organ dysfunction 6.
• Mycophenolate Mofetil (MMF) has been shown to halt the progression of rapidly progressive diffuse cutaneous SSc, but optimal MMF therapy duration has not been established, and recurrence of severe skin involvement can occur after MMF discontinuation or dose reduction 7.

Treatment Considerations

• The clinical management of patients with SSc remains a challenge, and treatment approaches must take into account the organs involved, the level of disease activity, and the disease stage 3, 4, 6.
• Careful risk-benefit considerations are necessary when considering HSCT, and additional research is needed to determine the effectiveness and adverse effects of non-myeloablative non-selective HSCT in the treatment of SSc 5.