What is the primary treatment approach for pulmonary fibrosis?

Treatment for Pulmonary Fibrosis

Antifibrotic medications (pirfenidone and nintedanib) are the primary treatment approach for pulmonary fibrosis, particularly idiopathic pulmonary fibrosis (IPF), as they slow disease progression by reducing the rate of decline in lung function.

First-Line Pharmacological Treatment

• Pirfenidone is recommended for patients with mild-to-moderate IPF (defined as FVC > 50% predicted and DLCO > 35% predicted) 1
• Nintedanib is conditionally recommended for IPF and for progressive pulmonary fibrosis (PPF) in patients with fibrotic interstitial lung disease (ILD) 1, 2
• Both medications have been shown to reduce the rate of forced vital capacity (FVC) decline over time 2, 3, 4
• Pirfenidone works through anti-inflammatory, antioxidative, and antiproliferative effects 1, 2
• Nintedanib blocks multiple tyrosine kinase receptors involved in fibrogenesis pathways 2, 5

Dosing and Administration

• Pirfenidone recommended daily maintenance dosage is 801 mg three times daily (2,403 mg/day total), taken with food 6
• Pirfenidone requires initial dose titration over 14 days to minimize side effects 6
• Treatment should be initiated and supervised by physicians experienced in the diagnosis and management of IPF 1, 2
• Regular monitoring of liver function is required for both medications 2, 6

Managing Side Effects

• Common side effects of pirfenidone include:
  • Photosensitivity (patients must avoid UV exposure) 1
  • Gastrointestinal symptoms (nausea, stomach discomfort) 1, 2
  • Fatigue and anorexia 1, 2
  • Elevated liver enzymes 2, 6
• Dose modifications or temporary interruptions may be necessary to manage adverse reactions 6
• Taking medication with food and gradual dose titration can help reduce gastrointestinal side effects 2

Treatment Monitoring

• Regular assessment of pulmonary function tests (FVC, DLCO) every 3-6 months to monitor disease progression and treatment response 1, 2
• Consider high-resolution computed tomography (HRCT) annually if clinical suspicion of worsening or increased risk of lung cancer 1
• Monitor liver function tests monthly for the first 6 months of treatment and every 3 months thereafter 2

Treatments to Avoid

• Triple therapy with prednisone, azathioprine, and N-acetylcysteine should NOT be used in IPF as it increases mortality 2
• Corticosteroid monotherapy is not recommended for IPF except for acute exacerbations 1, 2
• Avoid oral anti-vitamin K anticoagulants for treating IPF 1
• Do not use colchicine, cyclosporine A, interferon-γ-1b, etanercept, bosentan, macitentan, or ambrisentan 1

Non-Pharmacological Management

• Oxygen supplementation for patients with hypoxemia 1, 2
• Pulmonary rehabilitation for patients with limited exercise capacity 1, 2
• Lung transplantation should be considered for eligible patients with severe or progressive disease 1, 2
• Annual influenza and pneumococcal vaccinations 2
• Palliative care for symptom management (cough, dyspnea, anxiety) 1

Special Considerations

• For acute exacerbations of IPF, high-dose corticosteroids are commonly used 1
• Progressive pulmonary fibrosis (PPF) is defined by at least two of: worsening respiratory symptoms, physiological decline (FVC >5% or DLCO >10% within 1 year), or radiological progression 1
• Mechanical ventilation is generally not recommended for respiratory failure due to IPF progression 1

Treatment Limitations and Caveats

• Current antifibrotic therapies only slow disease progression but do not stop or reverse fibrosis 7, 4, 8
• Optimal duration of therapy is unknown, and it's unclear how long treatment effects endure with ongoing therapy 1
• Limited data exists for patients with severe disease (FVC < 50% or DLCO < 35%) 1
• Treatment decisions should involve shared decision-making, considering potential adverse effects and cost 1