Chronic progressive fibrosing interstitial pneumonia of unknown cause with a usual interstitial pneumonia (UIP) pattern.
Clinical features
- • Progressive exertional dyspnoea
- • Dry cough
- • Fine bibasal Velcro crackles
- • Finger clubbing
Investigations
- • HRCT: subpleural basal reticulation, honeycombing, traction bronchiectasis
- • Full lung function with transfer factor (restrictive, low DLCO)
- • Autoimmune and hypersensitivity screen to exclude mimics
- • Multidisciplinary ILD meeting
Management
- • Antifibrotics: pirfenidone or nintedanib
- • Ambulatory oxygen, pulmonary rehabilitation
- • GORD treatment, vaccination, smoking cessation
- • Early transplant referral; palliative care for cough and breathlessness
Clinical pearls
- • Steroids and immunosuppression worsen outcomes in IPF (PANTHER-IPF)
- • Acute exacerbation carries >50% inpatient mortality
Related in WardRound
Educational — verify locally.
