What is the treatment for interstitial lung disease (ILD)?

From the Guidelines

Treatment for interstitial lung disease (ILD) should prioritize immunosuppressive medications, such as mycophenolate mofetil or azathioprine, as first-line therapy, with nintedanib considered as an additional option for patients with systemic sclerosis-associated ILD, as recommended by the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline 1. The primary goal of treatment is to slow disease progression, reduce inflammation, and improve quality of life.

• For patients with systemic sclerosis-associated ILD, nintedanib (150 mg twice daily) can be considered as a first-line treatment option, in addition to immunosuppressive medications, as it has been shown to slow lung function decline 1.
• Supportive care is essential, including:
  • Supplemental oxygen for those with low blood oxygen levels
  • Pulmonary rehabilitation to improve exercise capacity
  • Vaccination against respiratory infections
  • Treating underlying conditions like GERD with proton pump inhibitors to prevent exacerbations
• For severe, end-stage disease, lung transplantation may be considered.
• Regular monitoring of lung function, oxygen levels, and medication side effects is crucial for optimal management. The 2023 ACR/CHEST guideline conditionally recommends against leflunomide, methotrexate, TNFi, and abatacept as first-line ILD treatment options, due to lack of evidence or potential harm 1. Pulmonary rehabilitation has been shown to result in meaningful short-term benefits in patients with ILD, including improvements in functional exercise tolerance, dyspnea, and quality of life 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 Study drug was administered three times daily with food for a minimum of 72 weeks Patients continued on treatment until the last patient completed 72 weeks of treatment, which included observations to approximately 120 weeks of study treatment The primary endpoint was the change in percent predicted forced vital capacity (%FVC) from baseline to study end, measured at 52 weeks in Study 1, and at 72 weeks in Studies 2 and 3

Treatment of Interstitial Lung Disease:

• Pirfenidone is used to treat Idiopathic Pulmonary Fibrosis (IPF), a type of interstitial lung disease.
• The recommended dosage is 2,403 mg/day, administered three times daily with food.
• Treatment should be continued for a minimum of 72 weeks.
• Key benefits of pirfenidone include:
  • Reduction in the decline of forced vital capacity (FVC)
  • Slowdown of disease progression
• Important considerations:
  • Pirfenidone is not a cure for IPF, but rather a treatment to slow down disease progression.
  • Patients should be monitored regularly for adverse reactions and disease progression.
  • The efficacy and safety of pirfenidone have been established in patients with IPF, but its use in other types of interstitial lung disease has not been extensively studied 2, 2.

From the Research

Treatment Options for Interstitial Lung Disease

• Antifibrotic therapy with nintedanib or pirfenidone slows annual forced vital capacity (FVC) decline by approximately 44% to 57% in individuals with idiopathic pulmonary fibrosis (IPF), scleroderma associated ILD, and in those with progressive pulmonary fibrosis of any cause 3
• Immunomodulatory therapy, such as tocilizumab, rituximab, and mycophenolate mofetil, may slow decline or even improve FVC at 12-month follow-up for connective tissue disease-associated ILD 3
• Structured exercise therapy reduces symptoms and improves 6-minute walk test distance in individuals with dyspnea 3
• Oxygen reduces symptoms and improves quality of life in individuals with ILD who desaturate below 88% on a 6-minute walk test 3
• Lung transplant may improve symptoms and resolve respiratory failure in patients with end-stage ILD, with a median survival of 5.2 to 6.7 years compared to less than 2 years in patients with advanced ILD who do not undergo lung transplant 3

Antifibrotic Medications

• Pirfenidone and nintedanib are antifibrotic medications approved for IPF treatment, which have been shown to reduce the rate of decline in forced vital capacity and the risk of acute exacerbation among patients with IPF 4, 5, 6
• Nintedanib has demonstrated efficacy in slowing disease progression in patients with interstitial lung diseases other than IPF 4
• Results on the use of pirfenidone in interstitial lung diseases other than IPF should be interpreted with caution due to trial limitations 4

Management and Diagnosis

• Early detection of ILD is important, as some forms can progress to pulmonary fibrosis 7
• A prompt and accurate diagnosis of the underlying cause is imperative to institute appropriate management 7
• The clinical course of ILD varies considerably, largely determined by the underlying cause, and while some forms remain essentially untreatable, many forms respond well to treatment 7