What are the treatment options for Systemic Sclerosis (SSc) associated interstitial lung disease?

From the Guidelines

Treatment of scleroderma-associated lung disease should primarily focus on immunosuppressive therapy, with mycophenolate and cyclophosphamide being considered as first-line options, and nintedanib as a first-line option specifically for SSc-ILD, as recommended by the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline 1. The treatment approach for scleroderma-associated lung disease involves a combination of immunosuppressive therapies and antifibrotic agents.

• Mycophenolate and cyclophosphamide are considered first-line options for SARD-ILD, including SSc-ILD, due to their immunosuppressive properties 1.
• Nintedanib, an antifibrotic agent, is specifically recommended as a first-line option for SSc-ILD, given its ability to slow lung function decline 1.
• Other treatment options, such as rituximab and tocilizumab, may be considered for patients with progressive interstitial lung disease despite initial treatments 1.
• Pulmonary arterial hypertension associated with scleroderma requires specific vasodilator therapies, including endothelin receptor antagonists, phosphodiesterase-5 inhibitors, or prostacyclin analogs 1.
• Supportive care, including oxygen therapy, pulmonary rehabilitation, vaccination against respiratory infections, and prompt treatment of gastroesophageal reflux, is essential to maintain quality of life and prevent further lung damage 1. The 2023 ACR/CHEST guideline provides conditional recommendations for the treatment of SARD-ILD, including SSc-ILD, based on the available evidence and expert opinion 1.
• The guideline recommends against the use of glucocorticoids as a first-line treatment for SSc-ILD, due to the potential for adverse effects and limited efficacy 1.
• The use of nintedanib and pirfenidone as first-line treatments for SARD-ILD is also conditionally recommended against, except for SSc-ILD, where nintedanib is recommended 1.
• The guideline also provides recommendations for the treatment of SARD-ILD progression despite first-line treatment, including the use of mycophenolate, rituximab, cyclophosphamide, and nintedanib as treatment options 1.

From the Research

Treatment Options for Scleroderma Associated Lung Disease

• The treatment options for scleroderma associated lung disease include immunosuppressant drugs, anti-fibrotic agents, and lung transplantation 2.
• Mycophenolate mofetil and cyclophosphamide are commonly used as first line therapy for scleroderma-related interstitial lung disease (SSc-ILD) 2, 3.
• A study comparing mycophenolate mofetil and cyclophosphamide found that both treatments resulted in significant improvements in lung function over a 2-year period, but mycophenolate mofetil was better tolerated and associated with less toxicity 3.
• The use of mycophenolate is recommended for the treatment of patients with SSc-ILD, and further research is needed to determine the safety and efficacy of pirfenidone and the combination of pirfenidone plus mycophenolate 4.
• Other treatment options that may be considered include rituximab, tocilizumab, nintedanib, and the combination of nintedanib plus mycophenolate 4.
• Lung transplantation may be an option for selected patients with SSc-ILD, but meticulous assessment for co-morbidities is crucial 2.
• A case study of a 50-year-old woman with limited scleroderma and interstitial lung disease found that treatment with mycophenolate mofetil and low-dose prednisone, followed by IV treprostinil, improved her symptoms and lung function 5.

Immunomodulatory Therapy

• Cyclophosphamide has been shown to be effective in improving lung function, functional status, and health-related quality of life in patients with SSc-ILD, but it can cause significant toxicity 6.
• Mycophenolate mofetil has a better tolerability profile than cyclophosphamide and is often preferred as a first-line treatment for SSc-ILD 3.

Future Directions

• Further research is needed to determine the safety and efficacy of new treatments for SSc-ILD, including anti-fibrotic agents and biologic agents 2, 4.
• The development of evidence-based clinical practice guidelines for the treatment of SSc-ILD will help to inform clinical decision-making and improve patient outcomes 4.