What is the treatment for lung fibrosis?

From the Guidelines

The treatment for lung fibrosis should focus on slowing disease progression, relieving symptoms, and improving quality of life, with antifibrotic medications like nintedanib (150mg twice daily) being a primary pharmacological treatment, as evidenced by the most recent and highest quality study 1.

Treatment Overview

The goal of treatment is to slow the progression of lung fibrosis, manage symptoms, and improve quality of life. This can be achieved through a combination of pharmacological and non-pharmacological interventions.

Pharmacological Treatments

• Antifibrotic therapy: Nintedanib (150mg twice daily) is a recommended treatment for lung fibrosis, as it has been shown to slow the decline in lung function 1.
• Immunosuppression: Medications like mycophenolate mofetil (MMF) and azathioprine may be used to treat underlying conditions that contribute to lung fibrosis, such as systemic sclerosis 1.

Non-Pharmacological Interventions

• Supplemental oxygen therapy: Oxygen therapy is prescribed when blood oxygen levels fall below normal, starting with exertional use and potentially progressing to continuous use as needed.
• Pulmonary rehabilitation: Programs combining exercise training, breathing techniques, and education are essential components of management, although the evidence for their effectiveness is low-quality 1.
• Lifestyle modifications: Smoking cessation, avoiding lung irritants, maintaining good nutrition, and staying physically active within limitations are crucial for managing lung fibrosis.

Symptom Management

• Cough suppressants: May be used to manage cough symptoms.
• Pulmonary vasodilators: May be used to treat associated pulmonary hypertension.
• Vaccinations: Against respiratory infections, such as influenza and pneumococcal infections, are important for preventing exacerbations.

Severe Cases

• Lung transplantation: May be considered for eligible patients with severe lung fibrosis unresponsive to medication, as recommended by strong evidence, albeit of low quality 1.

From the FDA Drug Label

Pirfenidone is indicated for the treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone is a pyridone indicated for the treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone is a prescription medicine used to treat people with a lung disease called idiopathic pulmonary fibrosis (IPF).

The treatment for lung fibrosis, specifically idiopathic pulmonary fibrosis (IPF), is pirfenidone.

• Pirfenidone is a prescription medicine used to treat people with IPF 2, 2, 2.
• It is not known if pirfenidone is safe and effective in children.
• Patients should take pirfenidone exactly as their doctor tells them to take it, with food at the same time each day.

From the Research

Treatment for Lung Fibrosis

The treatment for lung fibrosis, also known as pulmonary fibrosis, typically involves the use of antifibrotic medications.

• Pirfenidone and Nintedanib are two specific drugs that have been shown to be effective in treating idiopathic pulmonary fibrosis (IPF) and other forms of pulmonary fibrosis 3, 4, 5, 6, 7.
• These medications work by reducing the rate of decline in forced vital capacity (FVC), which is a measure of lung function, and can help to slow the progression of the disease 3, 4, 6.

Efficacy of Pirfenidone and Nintedanib

• Studies have demonstrated that both Pirfenidone and Nintedanib can reduce the rate of decline in FVC and improve outcomes for patients with IPF and other forms of pulmonary fibrosis 3, 4, 5, 6.
• A systematic review and meta-analysis found that antifibrotic therapy, including Pirfenidone and Nintedanib, can reduce the rate of decline in FVC and improve survival in patients with IPF and non-IPF pulmonary fibrosis 6.
• Another study found that combination therapy with Pirfenidone and Nintedanib can reduce the rate of decline in FVC and improve outcomes for patients with IPF, although it may increase the risk of certain side effects such as diarrhea 5.

Side Effects and Safety

• Both Pirfenidone and Nintedanib can cause side effects, including gastrointestinal symptoms such as diarrhea, dyspepsia, and vomiting, as well as photosensitivity and skin rashes 3, 5.
• The safety and efficacy of combination therapy with Pirfenidone and Nintedanib have been evaluated in several studies, and while it may increase the risk of certain side effects, it can also improve outcomes for patients with IPF 5.
• Patients should be closely monitored for side effects and adjusted as needed to minimize the risk of adverse events 3, 5.