A process in which a progressive interstitial lung disease causing permanent lung scarring is managed with HRCT, multidisciplinary evaluation and antifibrotic medications.
Indication
- Progressive shortness of breath and dry cough (typically over 50 years of age)
- Fine inspiratory crackles (Velcro rales) heard on lung auscultation
- Clubbing of the fingertips
- Restrictive pattern and reduced DLCO on pulmonary function testing
- Suspicion of an unexplained UIP (usual interstitial pneumonia) pattern
- Need to exclude other interstitial lung diseases
- Family history of pulmonary fibrosis
Preparation
- A detailed history of occupational, environmental and medication exposures is taken
- Smoking history is reviewed
- Blood tests for screening autoimmune diseases
- Removal of metal items before HRCT
- Fasting for 6-8 hours if bronchoscopy is planned
How it's performed
- High-resolution chest CT (HRCT) to assess the UIP pattern (subpleural, basal-predominant honeycombing)
- Additional evaluation with lung MRI in selected cases
- Pulmonary function tests, DLCO and 6-minute walk test
- Exclusion of connective tissue disease with autoimmune markers (ANA, rheumatoid factor)
- Bronchoalveolar lavage or surgical lung biopsy when needed
- Definitive diagnosis through a multidisciplinary board (pulmonology, radiology, pathology)
Post-procedure
- Antifibrotic therapy: nintedanib or pirfenidone — slows the rate of disease progression
- Monitoring of liver enzymes and gastrointestinal side effects when starting medication
- Pulmonary function tests and 6-minute walk test every 3-6 months
- Annual radiologic follow-up with HRCT
- Pulmonary rehabilitation, evaluation of oxygen needs, and vaccination (influenza, pneumococcal, COVID-19)
Risks
- Nausea, diarrhea and elevated liver enzymes due to antifibrotic medications (nintedanib)
- Skin photosensitivity and increased sensitivity to light with pirfenidone
- Acute exacerbation of the disease — high mortality risk
- Development of chronic respiratory failure
- Lung transplantation may be considered in selected patients
FAQ
Can IPF be cured completely?
IPF is a chronic and progressive disease. Antifibrotic medications slow the rate of disease progression but do not reverse existing damage.
Does smoking worsen IPF?
Yes. Smoking cessation has an important positive effect on the course of the disease.
Can I exercise?
A pulmonary rehabilitation program improves respiratory capacity and quality of life; regular moderate-intensity exercise is recommended.
Who is a candidate for lung transplantation?
In advanced IPF cases without other medical contraindications, lung transplantation is considered for selected patients.
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