Treatment of Pulmonary Fibrosis
For patients with idiopathic pulmonary fibrosis (IPF) or progressive pulmonary fibrosis (PPF), initiate antifibrotic therapy with either pirfenidone or nintedanib as first-line pharmacological treatment, combined with oxygen supplementation if hypoxemic and pulmonary rehabilitation. 1, 2
Pharmacological Treatment Algorithm
First-Line Antifibrotic Therapy
Pirfenidone is indicated for treatment of IPF, particularly in patients with mild-to-moderate disease (FVC >50% predicted and DLCO >35% predicted). 1, 2 The medication works through anti-inflammatory, antioxidative, and antiproliferative mechanisms. 1 Standard dosing is 2,403 mg/day divided into three doses taken with food. 2
Nintedanib is recommended for progressive pulmonary fibrosis in patients who have failed standard management for fibrotic interstitial lung disease, blocking pathways involved in fibrogenesis. 1 This is particularly appropriate for non-IPF progressive fibrotic ILD. 1
Managing Adverse Effects
For pirfenidone, common side effects include nausea, rash, fatigue, diarrhea, photosensitivity, and elevated liver enzymes. 1 Manage these through:
- Gradual dose titration 1
- Taking medication with food 1
- Avoiding sun exposure 1
- Monthly liver function monitoring for first 6 months, then every 3 months 1
For nintedanib, expect diarrhea, nausea, abdominal pain, vomiting, and elevated liver enzymes. 1 Address through dose reduction and temporary treatment interruption as needed. 1
What NOT to Use
Avoid combined corticosteroids and immunosuppressants - this approach has demonstrated increased mortality in IPF. 1 The triple therapy with prednisone, azathioprine, and N-acetylcysteine is contraindicated as it increases mortality. 1 Corticosteroid monotherapy is no longer recommended except for incapacitating cough or acute exacerbations. 1 Ambrisentan is contraindicated in IPF. 1
Non-Pharmacological Management
Oxygen Therapy
Provide supplemental oxygen for patients with hypoxemia, particularly those with oxygen saturation <88% during 6-minute walk testing. 3 Measure oxygen saturation at rest and with exertion at every visit regardless of symptoms. 3
Pulmonary Rehabilitation
Implement pulmonary rehabilitation programs to improve exercise capacity and quality of life. 3, 1 This carries a weak recommendation but provides meaningful benefit. 3
Vaccinations
Administer annual influenza and pneumococcal vaccinations to all patients. 1
Monitoring Protocol
Frequency and Parameters
Monitor patients every 3-6 months or sooner if clinically indicated. 3 At each visit, assess:
- Pulmonary function testing (FVC and DLCO) 3
- 6-minute walk test 3
- Oxygen saturation at rest and with exertion 3
- Respiratory symptoms (dyspnea, cough) 3
Disease Progression Criteria
Progressive disease is defined by a decline in FVC >10% or decline in DLCO >15% over monitoring periods. 3 Smaller declines (5-10% FVC) may represent progression but require cautious interpretation due to test variability. 3 The average annual FVC decline in untreated IPF is approximately 0.2 liters. 3
Radiological Monitoring
Consider annual HRCT if clinical suspicion of worsening or risk of lung cancer exists. 3 Obtain HRCT if concern for acute exacerbation arises. 3
Lung Transplantation Evaluation
Refer patients at increased risk of mortality for lung transplantation evaluation at the time of diagnosis. 3 This is the only intervention proven to improve survival. 4 Consider transplantation for patients aged <65 years with severe or worsening disease. 1
Acute Exacerbation Management
Corticosteroids are an appropriate treatment option for acute exacerbations (weak recommendation). 3 However, mechanical ventilation is not recommended for the majority of patients with respiratory failure due to disease progression (weak recommendation against). 3
Palliative Care Integration
Integrate palliative care early to manage symptoms including cough, dyspnea, and anxiety. 3 Focus on reducing symptoms and providing comfort rather than treating the underlying disease. 3 Discuss advanced directives in the ambulatory setting. 3
Comorbidity Screening
Evaluate and treat existing comorbidities including:
- Pulmonary hypertension 3
- Gastroesophageal reflux 3
- Obstructive sleep apnea 3
- Lung cancer (15% increased risk) 4
- Coronary artery disease 3
- Pulmonary embolism 3
Clinical Trial Enrollment
Inform all patients about available clinical trials for possible enrollment at all stages of disease. 3 This provides access to emerging therapies and contributes to advancing treatment options.
Special Considerations
For patients with severe disease (FVC <50% or DLCO <35%), treatment may still be considered on an individual basis, though clinical trial data is limited. 1 Median survival without treatment ranges from 2-4 years after diagnosis. 3, 4, 5 With current antifibrotic therapy, these agents slow but do not halt disease progression. 6, 5