From the Guidelines
Pulmonary fibrosis treatment should prioritize antifibrotic medications, such as pirfenidone and nintedanib, as they have demonstrated benefits in slowing disease progression and improving quality of life 1.
Treatment Overview
Pulmonary fibrosis treatment typically involves a combination of antifibrotic medications, supportive care, and in some cases, lung transplantation. The goal of treatment is to slow disease progression, improve symptoms, and enhance quality of life.
Antifibrotic Medications
The two main FDA-approved antifibrotic medications are pirfenidone (Esbriet) and nintedanib (Ofev).
- Pirfenidone is typically started at 267 mg three times daily for one week, then increased to 534 mg three times daily for one week, and finally to the maintenance dose of 801 mg three times daily with food.
- Nintedanib is usually prescribed as 150 mg twice daily with food, though dose reduction to 100 mg twice daily may be necessary if side effects occur.
Supportive Care
Supportive care measures include:
- Supplemental oxygen therapy when oxygen saturation falls below 88% at rest or with exertion
- Pulmonary rehabilitation programs to improve exercise capacity and quality of life
- Vaccination against influenza and pneumococcal pneumonia to prevent respiratory infections
- Addressing gastroesophageal reflux disease if present, as it can worsen fibrosis
Treatment Considerations
Treatment should be individualized based on the patient's specific needs and preferences. The use of corticosteroids is no longer recommended for the treatment of IPF, except to alleviate incapacitating cough or to treat acute exacerbation of IPF 1. Nintedanib may be considered for the treatment of progressive pulmonary fibrosis in patients who have failed standard management for fibrotic ILD, other than IPF 1.
Recent Guidelines
Recent guidelines, such as the 2022 ATS/ERS/JRS/ALAT clinical practice guideline, provide updated recommendations for the diagnosis and management of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis in adults 1. Pirfenidone has been shown to increase progression-free survival in IPF patients, making it a valuable treatment option 1.
From the FDA Drug Label
Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF). The recommended daily maintenance dosage of pirfenidone is 801 mg three times daily for a total of 2,403 mg/day.
Pirfenidone is used to treat idiopathic pulmonary fibrosis (IPF). The recommended dosage is 801 mg three times daily for a total of 2,403 mg/day 2.
- Key points:
- Pirfenidone is indicated for IPF treatment
- Daily maintenance dosage: 801 mg three times daily
- Total daily dosage: 2,403 mg/day
- Dosage titration: The dosage should be titrated over a 14-day period to the full maintenance dosage of 2,403 mg/day 2.
From the Research
Treatment Options for Pulmonary Fibrosis
- Pirfenidone and nintedanib are two antifibrotic therapies approved for the treatment of idiopathic pulmonary fibrosis (IPF) 3, 4.
- These drugs have been shown to slow the decline in lung function and reduce the risk of acute respiratory deteriorations 4.
- Pirfenidone has been found to provide a meaningful clinical effect on reductions in the decrease in forced vital capacity (FVC), six-minute walk test (6MWT) distance, and mortality, and improves the progression-free survival in IPF patients with mild-to-moderate disease 3.
- Nintedanib has been demonstrated to be effective in slowing disease progression in patients with interstitial lung diseases other than IPF 5.
Efficacy and Safety of Antifibrotic Therapies
- Pirfenidone and nintedanib have been found to be well-tolerated, with the most common side-effects being gastrointestinal discomfort and photosensitivity 3.
- The use of antifibrotic therapies has been associated with improved long-term survival in patients with IPF, particularly when treatment is continued after hospital discharge 6.
- However, antifibrotic medications do not appear to directly improve 30-day mortality during or after respiratory-related hospitalizations 6.
Future Directions for Treatment
- There is a need for more effective and targeted therapies that can halt the progression of IPF, as current treatments cannot completely stop the loss of lung function 7.
- Novel strategies targeting key mechanisms involved in the pathogenesis of IPF, such as JAK/STAT, TGF-β/Smad, Wnt/β-catenin, PI3K, and JNK1 signaling pathways, may lead to the development of more potent and selective therapeutic agents 7.