What is the treatment for Progressive Fibrosing Interstitial Lung Disease (ILD)?

From the Guidelines

The treatment for Progressive Fibrosing Interstitial Lung Disease (ILD) should prioritize nintedanib (Ofev) at 150mg twice daily, as it has shown to slow disease progression by inhibiting fibrotic pathways, although it does not reverse existing damage, as supported by the most recent and highest quality study 1.

Key Considerations

• Nintedanib is recommended for the treatment of Progressive Pulmonary Fibrosis (PPF) in patients who have failed standard management for fibrotic ILD, other than IPF, with a conditional recommendation and low-quality evidence 1.
• The use of pirfenidone is still under research for its efficacy, effectiveness, and safety in non-IPF ILD manifesting PPF, and its recommendation is pending further studies 1.
• Corticosteroids and immunosuppressants may be used for inflammatory components, but their use should be individualized based on the specific ILD subtype, disease severity, and patient factors.

Treatment Approach

• Nintedanib should be considered as the first-line treatment for Progressive Fibrosing ILD, especially in patients with a UIP pattern of ILD, as suggested by the 2023 ACR/CHEST guideline 1.
• Supportive care, including supplemental oxygen, pulmonary rehabilitation programs, and vaccination against respiratory infections, is essential to improve quality of life and slow disease progression.
• Lung transplantation may be considered for eligible patients in advanced cases.

Important Outcomes

• Mortality and disease progression (determined by change in FVC) are critical outcomes to consider when evaluating the effectiveness of treatment.
• Lung function, respiratory symptoms, and adverse events should also be monitored to adjust treatment accordingly.

Individualized Treatment

• Treatment should be tailored to the specific ILD subtype, disease severity, and patient factors, taking into account the potential benefits and risks of each treatment option.
• Early diagnosis and treatment initiation are crucial to impact disease progression and quality of life, although the condition cannot be cured completely.

From the FDA Drug Label

The FDA drug label does not answer the question.

From the Research

Treatment Options for Progressive Fibrosing Interstitial Lung Disease (ILD)

The treatment for Progressive Fibrosing Interstitial Lung Disease (ILD) involves a combination of pharmacological and non-pharmacological approaches. Some of the key treatment options include:

• Antifibrotic therapy with nintedanib or pirfenidone, which has been shown to slow lung function decline in patients with IPF and PF-ILD 2, 3, 4
• Immunosuppression, which may be used in certain subtypes of PF-ILD, such as connective tissue disease ILD and hypersensitivity pneumonitis 2
• Inhaled treprostinil, a novel therapy that has been shown to improve exercise tolerance in patients with PF-ILD and concomitant pulmonary hypertension 2
• Lung transplantation, which is the only curative therapy for PF-ILD and may be considered in suitable patients 2
• Supportive care, including oxygen therapy and treatment of comorbid conditions, which is an essential aspect of PF-ILD management 2, 5

Pharmacological Treatment

Pharmacological treatment for PF-ILD is primarily focused on slowing disease progression and improving symptoms. Key pharmacological treatments include:

• Nintedanib, which has been shown to slow lung function decline in patients with IPF and PF-ILD 2, 3, 4
• Pirfenidone, which has also been shown to slow lung function decline in patients with IPF and may be used in patients with PF-ILD 2, 3
• Antifibrotic therapies, which may be used in combination with immunosuppressant drugs to address both the fibrotic and inflammatory components of ILD 4

Non-Pharmacological Treatment

Non-pharmacological treatment for PF-ILD is focused on improving symptoms and quality of life. Key non-pharmacological treatments include:

• Oxygen therapy, which may be used to improve oxygenation and reduce symptoms in patients with PF-ILD 2, 5
• Supportive care, including pulmonary rehabilitation and treatment of comorbid conditions, which is an essential aspect of PF-ILD management 2, 5
• Lung transplantation, which may be considered in suitable patients with advanced PF-ILD 2