Progressive pulmonary fibrosis (PPF)
- Treat the underlying interstitial lung disease when possible
- Document objective progression despite appropriate management
- Nintedanib slows FVC decline in progressive fibrosing ILD
- Antifibrotic choice depends on disease subtype
- Pulmonary rehab/oxygen + transplant referral may be needed
- Avoid reflex immunosuppression in every fibrotic phenotype
Use at a glance
- Treat the underlying interstitial lung disease when possible
Escalation / refractory disease
- Nintedanib slows FVC decline in progressive fibrosing ILD
- Pulmonary rehab/oxygen + transplant referral may be needed
Choosing treatment
- Document objective progression despite appropriate management
- Antifibrotic choice depends on disease subtype
Safety / pitfalls
- Avoid reflex immunosuppression in every fibrotic phenotype
Self-check
- Which drug slows FVC decline in progressive fibrosing ILD?
Nintedanib. - Should every fibrotic phenotype receive reflex immunosuppression?
No.
Drugs used for this (2)
All clinical uses · Open Progressive pulmonary fibrosis (PPF) in the interactive console ↗