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
