From the Guidelines
Treatment for chronic interstitial lung disease (ILD) should be tailored to the specific ILD subtype, with a focus on slowing disease progression, relieving symptoms, and improving quality of life. The cornerstone of therapy is anti-inflammatory and antifibrotic medications. For people with SARD-ILD other than SSc-ILD, glucocorticoids are conditionally recommended as a first-line ILD treatment 1. However, for people with SSc-ILD, glucocorticoids are strongly recommended against as a first-line ILD treatment 1. Immunosuppressants such as mycophenolate mofetil, azathioprine, rituximab, and cyclophosphamide are conditionally recommended as first-line ILD treatment options for people with SARD-ILD 1. Antifibrotic medications like nintedanib are conditionally recommended for people with SSc-ILD, but not for those with SjD-ILD, IIM-ILD, and MCTD-ILD 1. Supportive care includes supplemental oxygen for hypoxemia, pulmonary rehabilitation to improve exercise capacity, vaccination against respiratory infections, and treatment of gastroesophageal reflux. Smoking cessation and avoidance of environmental triggers are essential. For end-stage disease, lung transplantation may be considered in appropriate candidates. Regular monitoring of lung function, oxygen levels, and medication side effects is necessary to adjust therapy accordingly 1.
Some key points to consider in the treatment of ILD include:
- The use of immunosuppressants and antifibrotic medications to slow disease progression and improve symptoms
- The importance of supportive care, including supplemental oxygen and pulmonary rehabilitation
- The need for regular monitoring of lung function and medication side effects to adjust therapy accordingly
- The consideration of lung transplantation for end-stage disease
- The importance of tailoring treatment to the specific ILD subtype, as response varies significantly between different forms of the disease 1.
Overall, the treatment of ILD requires a comprehensive and multidisciplinary approach, taking into account the specific needs and characteristics of each patient. The goal of treatment is to slow disease progression, relieve symptoms, and improve quality of life, while minimizing the risk of adverse effects and optimizing patient outcomes 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. In Study 1, the primary efficacy analysis for the change in %FVC from baseline to Week 52 demonstrated a statistically significant treatment effect of pirfenidone 2,403 mg/day (n=278) compared with placebo (n=277) A reduction in the mean decline in FVC (in mL) was observed in patients receiving pirfenidone 2,403 mg/day (-235 mL) compared to placebo (-428 mL) (mean treatment difference 193 mL) at Week 52
Pirfenidone is used for the treatment of Idiopathic Pulmonary Fibrosis (IPF), a type of chronic interstitial lung disease. The drug has been shown to slow the decline in forced vital capacity (FVC) in patients with IPF.
- The recommended dosage is 2,403 mg/day.
- The treatment effect was demonstrated in Study 1 and Study 2, but not in Study 3.
- Pirfenidone may be considered as a treatment option for patients with IPF who have a percent predicted FVC greater than or equal to 50% and a percent predicted diffusing capacity of the lungs for carbon monoxide (DLCO) greater than or equal to 35% at baseline 2.
From the Research
Treatment Options for Chronic 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
Pharmacological Treatment
- Nintedanib has been shown to slow disease progression in patients with interstitial lung diseases other than idiopathic pulmonary fibrosis, but caution should be exercised before prematurely endorsing and applying this therapy 4, 5
- Pirfenidone has been shown to reduce the rate of decline in forced vital capacity and the risk of acute exacerbation among patients with idiopathic pulmonary fibrosis, but results on its use in interstitial lung diseases other than idiopathic pulmonary fibrosis should be interpreted with caution 5
- Mycophenolate is recommended for the treatment of patients with systemic sclerosis-associated interstitial lung disease, and further research is needed into the safety and efficacy of pirfenidone and the combination of pirfenidone plus mycophenolate 6