What is the first line treatment for interstitial (infiltrative) pneumonia?

From the Guidelines

The first-line treatment for interstitial pneumonia should be mycophenolate, with consideration of nintedanib as an alternative, based on the most recent guidelines from the American College of Rheumatology (ACR) and American College of Chest Physicians (CHEST) 1.

Treatment Overview

The treatment approach for interstitial pneumonia involves a combination of immunosuppressive agents and anti-fibrotic medications. According to the 2023 ACR/CHEST guideline, mycophenolate is a preferred first-line therapy, with nintedanib being considered as an alternative, especially in patients with a usual interstitial pneumonia pattern on CT chest or significant fibrotic disease 1.

Key Considerations

• The decision to use nintedanib versus immunosuppression depends on the pace of progression and the amount of fibrotic disease 1.
• Short-term glucocorticoids may be used in some patients with disease flares or as a bridge when switching therapy, but there is a strong recommendation against long-term glucocorticoids due to their side effects 1.
• Other therapies such as rituximab, tocilizumab, and cyclophosphamide may be considered in specific clinical situations, but their use should be guided by the hierarchy established in the guideline and the individual patient's condition 1.

Monitoring and Adjustment

Treatment response should be regularly assessed through pulmonary function tests, imaging, and symptom improvement, typically every 3-6 months. If intolerance leads to suboptimal dosing of first-line therapy, switching to an alternative first-line therapy should be considered 1.

Additional Recommendations

Pulmonary rehabilitation and smoking cessation are essential components of management for patients with interstitial pneumonia. Oxygen therapy should be provided to maintain oxygen saturation above 88%, and bone protection with calcium and vitamin D supplements should be considered for patients on long-term glucocorticoids, although the guideline recommends against long-term use of glucocorticoids 1.

From the FDA Drug Label

The efficacy of pirfenidone was evaluated in patients with IPF in three phase 3, randomized, double-blind, placebo-controlled, multicenter trials (Studies 1,2, and 3) Study 1 was a 52-week trial comparing pirfenidone 2,403 mg/day (n=278) versus placebo (n=277) in patients with IPF. Study 2 and Study 3 were nearly identical to each other in design, with few exceptions, including an intermediate dose treatment arm in Study 2

First line treatment for Interstitial Pneumonia:

• Pirfenidone is used for the treatment of Idiopathic Pulmonary Fibrosis (IPF), a type of interstitial pneumonia.
• The recommended dosage is 2,403 mg/day, administered three times daily with food.
• Key benefits:
  • Reduction in the decline of forced vital capacity (FVC)
  • Lower proportion of patients with categorical declines in lung function
• Studies: 2, 2, and 2 support the use of pirfenidone for the treatment of IPF.

From the Research

First Line Treatment for Interstitial Pneumonia

• The first line treatment for interstitial pneumonia, specifically idiopathic pulmonary fibrosis (IPF), includes antifibrotic drugs such as pirfenidone and nintedanib, which have been shown to reduce lung function decline 3, 4, 5.
• For connective tissue disease-associated interstitial lung disease, immunomodulatory therapy, such as mycophenolate mofetil, may be used as first line treatment 4.
• Combination therapy with mycophenolate mofetil and prednisone has been associated with less disease progression in patients with interstitial pneumonia with autoimmune features 6.
• The choice of first line treatment may depend on the specific subtype of interstitial pneumonia, as well as the presence of any underlying autoimmune or connective tissue disease.

Treatment Goals and Outcomes

• The goal of first line treatment for interstitial pneumonia is to slow disease progression, improve symptoms, and improve quality of life 4, 5.
• Treatment outcomes may be measured by changes in forced vital capacity (FVC), as well as improvements in symptoms and quality of life 4, 5.
• Lung transplant may be considered for patients with advanced interstitial pneumonia who have not responded to first line treatment 4.

Specific Treatment Options

• Pirfenidone and nintedanib are antifibrotic drugs that have been shown to be effective in reducing lung function decline in patients with IPF 3, 4, 5.
• Mycophenolate mofetil is an immunomodulatory therapy that may be used as first line treatment for connective tissue disease-associated interstitial lung disease 4.
• Combination therapy with mycophenolate mofetil and prednisone may be associated with less disease progression in patients with interstitial pneumonia with autoimmune features 6.