Idiopathic pulmonary fibrosis: Difference between revisions
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* Chronic idiopathic interstitial lung disease characterized by a usual interstitial pneumonia on histopathology |
* Chronic idiopathic interstitial lung disease characterized by a usual interstitial pneumonia on histopathology |
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== Clinical |
== Clinical Manifestations == |
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* Fine velcro-like crackles |
* Fine velcro-like crackles |
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* CXR showing interstitial pattern |
* CXR showing interstitial pattern |
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* PFTs showing low DLCO and FVC (i.e. a restrictive pattern |
* PFTs showing low DLCO and FVC (i.e. a restrictive pattern) |
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* High-res CT scan showing a UIP pattern |
* High-res CT scan showing a UIP pattern |
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** Subpleural/basal |
** Subpleural/basal |
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** Reticulation |
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** reticulation |
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** Honeycombing |
** Honeycombing |
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** No incosistent features |
** No incosistent features |
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* Supplemental oxygen |
* Supplemental oxygen |
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* Medications |
* Medications |
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** Consider nintedanib and perfenidone |
** Consider [[nintedanib]] and [[perfenidone]] |
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** Avoid prednisone and azathioprine |
** Avoid prednisone and azathioprine |
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** NAC not helpful |
** NAC not helpful |
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Latest revision as of 22:25, 12 January 2022
See also Interstitial lung disease
Definition
- Chronic idiopathic interstitial lung disease characterized by a usual interstitial pneumonia on histopathology
Clinical Manifestations
- Fine velcro-like crackles
- Clubbing
Investigations
- CXR showing interstitial pattern
- PFTs showing low DLCO and FVC (i.e. a restrictive pattern)
- High-res CT scan showing a UIP pattern
- Subpleural/basal
- Reticulation
- Honeycombing
- No incosistent features
- If high-res CT indeterminate, consider biopsy (but 5% mortality from biopsy in ILD)
- CBC, electrolytes
- ANA/ENA, RF, CCP
- Precipitating antibodies
Management
- Smoking cessation
- Vaccines: seasonal influenza and pneumococcal (13 then 23)
- Pulmonary rehab
- Supplemental oxygen
- Medications
- Consider nintedanib and perfenidone
- Avoid prednisone and azathioprine
- NAC not helpful
- Trial of GERD treatment even if asymptomatic
- Early antibiotics for respiratory infections
