Table 2 Summary of available therapies for PF based on scientific society guidelines

First-line

Pirfenidone

Antifibrotic; anti-inflammatory; modulates growth factors (TGF-β, PDGF); inhibits fibroblast proliferation and collagen synthesis, and antioxidant.

Oral

Conditional recommendation for use in PF. Recommended if lung capacity is 50–80% expected.

Nintedanib

Tyrosine kinase inhibitor targeting PDGF, FGF, and VEGF receptors; inhibits fibroblast proliferation, migration, and differentiation.

Oral

Conditional recommendation for use in PF. Recommended if lung capacity is 50–80% expected.

Supportive care (Throughout)

Oxygen therapy

Supplemental oxygen to alleviate hypoxemia.

Inhalation

Recommended for patients with hypoxemia to improve breathing, exercise tolerance, and potentially reduce complications.

Pulmonary rehabilitation

Exercise training; breathing techniques; education; nutritional and psychological support.

Program-based

Strongly recommended to improve exercise capacity, reduce breathlessness, and enhance overall well-being.

Vaccinations (influenza, pneumococcal)

Prevention of respiratory infections.

Injection

Recommended to prevent exacerbations.

Smoking cessation

Eliminating a major risk factor for lung disease.

Behavioral/Pharmacological

Strongly recommended for current smokers.

Nutritional support & lifestyle

Maintaining a healthy diet; regular exercise within tolerance, and adequate rest.

Lifestyle modifications

Advised for managing symptoms and maintaining overall health.

Later-Stage/Severe Disease

Lung transplantation

Surgical replacement of diseased lungs with healthy donor lungs.

Surgical

Potential option for selected patients with severe and progressive PF who meet specific criteria. Early referral for evaluation is recommended.

Management of acute exacerbations

Corticosteroids

Anti-inflammatory.

Oral/Intravenous

Recommended for treating acute worsening of symptoms.