What are the treatment guidelines for interstitial lung disease, specifically idiopathic pulmonary fibrosis?

Treatment Guidelines for Interstitial Lung Disease

For patients with idiopathic pulmonary fibrosis (IPF), antifibrotic therapy with nintedanib is conditionally recommended as first-line treatment to slow disease progression and improve mortality outcomes. 1

Diagnosis and Classification

Interstitial lung disease (ILD) encompasses a heterogeneous group of disorders, with IPF being one of the most severe forms. Proper diagnosis requires:

• High-resolution computed tomography (HRCT) to identify radiological patterns:

  • UIP pattern (subpleural, basal predominance, reticular abnormality, honeycombing)
  • Possible UIP pattern
  • Inconsistent with UIP pattern

• Lung function tests to establish baseline and monitor progression:

  • Forced vital capacity (FVC)
  • Diffusing capacity for carbon monoxide (DLCO)

• In cases where HRCT is inconclusive, surgical lung biopsy or transbronchial lung cryobiopsy may be considered 1

Treatment Recommendations for IPF

Antifibrotic Therapy

1. Nintedanib (150mg twice daily):

   • Conditionally recommended for IPF 1
   • Reduces FVC decline by 44-57% 2
   • Significantly improves survival compared to no antifibrotic treatment 3

2. Pirfenidone:

   • Reduces FVC decline in IPF 4
   • Can be considered when nintedanib is not tolerated
   • Even reduced dosing may provide survival benefit when full dose cannot be tolerated 3

Treatments NOT Recommended for IPF

• Combination therapy with prednisone, azathioprine, and N-acetylcysteine: Strongly recommended against due to increased mortality risk 1

• Corticosteroid therapy: Not recommended as it has not been shown to improve survival and is associated with substantial morbidity 1, 2

• Ambrisentan: Contraindicated in IPF 1

• Antacid medications and antireflux surgery: Conditionally recommended against for treatment of IPF 1

Treatment for Progressive Pulmonary Fibrosis (non-IPF ILD)

Progressive pulmonary fibrosis (PPF) is defined as at least two of three criteria occurring within the past year with no alternative explanation 1:

1. Worsening symptoms
2. Radiological progression
3. Physiological progression

For non-IPF ILD with progressive fibrosis:

• Nintedanib: Conditionally recommended 1

• Pirfenidone: Additional research is recommended before routine use 1, 5

• For systemic autoimmune rheumatic disease-related ILD:

  • Glucocorticoids (except in SSc-ILD)
  • Mycophenolate, azathioprine, rituximab, and cyclophosphamide are conditionally recommended as first-line options 1
  • For SSc-ILD and MCTD-ILD, tocilizumab is conditionally recommended 1

Symptom Management

• Cough management:

  • Consider gabapentin for refractory cough 1
  • Speech pathology management 1
  • Opiates for patients with cough refractory to alternative therapies 1

• Oxygen therapy:

  • Long-term oxygen therapy for patients with resting hypoxemia
  • Ambulatory oxygen for exercise desaturation 2

Monitoring and Follow-up

• Pulmonary function tests every 3-6 months 2
• HRCT if suspicion of acute exacerbation or disease progression 2
• Liver function monitoring before and during treatment with antifibrotic medications 2
• Annual influenza and pneumococcal vaccination 2

Supportive Care

• Early integration of palliative care for symptom management 2
• Pulmonary rehabilitation to improve exercise capacity and quality of life 2
• Lung transplantation evaluation for eligible patients with severe or worsening disease 2

Practical Considerations

• Dose adjustment of antifibrotic medications may be effective at reducing side effects without compromising efficacy 6
• Most common side effects include:
  • Nintedanib: Diarrhea, nausea, elevated liver enzymes
  • Pirfenidone: Gastrointestinal symptoms, photosensitivity, skin rashes 7

The treatment approach should be guided by accurate diagnosis, disease progression, and careful monitoring of treatment response and side effects. Early initiation of antifibrotic therapy is critical to preserving lung function and improving outcomes in patients with IPF 6.