Treatment of Pulmonary Fibrosis
For idiopathic pulmonary fibrosis (IPF), initiate antifibrotic therapy with either nintedanib or pirfenidone immediately upon diagnosis to slow disease progression, combined with supportive care including oxygen supplementation and pulmonary rehabilitation. 1, 2
Pharmacological Treatment
First-Line Antifibrotic Therapy
The two FDA-approved antifibrotic medications should be started promptly:
Pirfenidone: Indicated for IPF treatment, dosed at 801 mg three times daily (2,403 mg/day total) with food 2
Nintedanib: Recommended by the American Thoracic Society to slow disease progression and reduce FVC decline 1
- Preferred for patients with mild-to-moderate IPF (FVC ≥50% predicted and DLCO ≥35% predicted) 1
Both medications slow but do not halt disease progression, with evidence suggesting improved life expectancy despite individual trials not being powered for mortality endpoints 3, 4
Therapies to AVOID
The combination of N-acetylcysteine, azathioprine, and prednisone ("triple therapy") is strongly contraindicated due to increased risk of harm and mortality. 1, 5
- Corticosteroids alone have weak evidence and are NOT recommended for routine IPF treatment 5
- Warfarin anticoagulation carries a strong contraindication due to increased mortality risk 5
- Azathioprine and cyclophosphamide as monotherapy or in combination with steroids are not recommended based on 2011 guidelines 5
Limited-Use Pharmacological Options
Non-Pharmacological Management
Oxygen Therapy
- Strongly recommended for patients with hypoxemia at rest 5, 1
- Prescribe supplemental oxygen when saturation falls below 88% during 6-minute walk test 5, 6
- Measure oxygen saturation at rest and with exertion at baseline and every 3-6 months 5
Pulmonary Rehabilitation
- Recommended to improve exercise capacity, walking distance, symptoms, and quality of life 5, 1, 6
- Particularly beneficial for patients with significant exercise limitation 1
- May not be feasible in advanced disease 5
Lung Transplantation
- Evaluate and list for transplantation at time of diagnosis if patient has increased mortality risk 5
- Consider when DLCO <39% predicted AND FVC decreased >10% over 6 months 5, 1
- Recommended for all IPF patients <65 years with severe or worsening disease 5, 1
- Improves survival in advanced-stage IPF 5
Vaccinations
Monitoring Strategy
Regular Assessment Schedule
- Monitor every 4-6 months or sooner if clinically indicated 5, 6
- Assess: dyspnea using established scales, pulmonary function tests (FVC, DLCO), oxygen saturation at rest and with exertion, 6-minute walk test 5
Disease Progression Criteria
Progressive disease is defined by ≥2 of the following within past year 5:
- Worsening respiratory symptoms
- Absolute FVC decline >5% predicted OR DLCO decline >10% predicted
- Radiological progression (increased traction bronchiectasis, new ground-glass opacity, new/increased reticulation, increased honeycombing) 5
Imaging Surveillance
- Annual HRCT if clinical suspicion of worsening or risk of lung cancer 5
- HRCT if concern for acute exacerbation 5
Management of Acute Exacerbations
Acute exacerbation is defined as acute worsening of dyspnea (<30 days) with new lung opacities after excluding infection, pulmonary embolism, and heart failure. 5
- High-dose corticosteroids are commonly used despite lack of controlled trial evidence 5
- Intravenous cyclophosphamide has been suggested as potentially beneficial 5
- Mechanical ventilation is NOT recommended for the majority of patients with respiratory failure due to IPF progression 5
- Exception: Consider as bridge to lung transplantation or if reversible cause identified 5
Critical Pitfalls to Avoid
When patients experience acute worsening, immediately evaluate for alternative etiologies before attributing to disease progression: 6
- Pulmonary embolism (most commonly missed)
- Pneumothorax
- Respiratory infection
- Aspiration
- Deep venous thrombosis
- Cardiac causes (heart failure, arrhythmia) 5
Do not delay treatment initiation. IPF has a median survival of only 3-4 years untreated, with almost linear FVC decline regardless of baseline severity 3, 7. Early treatment before irreversible fibrosis develops yields better response rates 5.