Idiopathic pulmonary fibrosis
Chronic progressive interstitial lung disease of unknown etiology with a characteristic usual interstitial pneumonia (UIP) pattern on high-resolution CT and/or lung biopsy. Predominantly in men over 60, smokers. Per ATS/ERS/JRS/ALAT 2022 and Spanish SEPAR 2023 first-line therapy includes the antifibrotics nintedanib (Ofev) or pirfenidone (Esbriet). They slow FVC decline by 50% but do not halt progression. Median survival without treatment 3–5 years, with therapy 5–7 years. Ultimate treatment – lung transplantation.
ICD-10 J84.12 drugs
Also known as: IPF, Cryptogenic fibrosing alveolitis
Drugs used for this condition
- ANintedanibFirst line · Tyrosine kinase inhibitorsFirst-line IPF therapy per ATS/ERS/JRS/ALAT 2022 and Spanish SEPAR 2023 – on par with pirfenidone. Dose 150 mg twice daily. INPULSIS-1 and INPULSIS-2 (NEJM 2014) reduced FVC decline by 50% (~100 mL/year vs 200 mL). Direct mortality benefit not proven, but meta-analyses show a downward trend. Duration – lifelong until intolerance or lung transplantation.
- APirfenidoneFirst line · Antifibrotic agentsFirst-line IPF therapy per ATS/ERS/JRS/ALAT 2022 and Spanish SEPAR 2023 – on par with nintedanib. Dose 801 mg three times daily (2403 mg/day) with meals. Titration: 267 mg three times daily in week 1, double in week 2, full dose by week 3. CAPACITY-1, CAPACITY-2, ASCEND (NEJM 2014) reduced FVC decline by 50%. Duration – lifelong until intolerance or transplantation.
How to read tiers A–F
Evidence tiers belong to the drug-indication pair, not to the drug as a whole: the same substance can be A for one condition and F for another.
- AStrong evidence – first or second line in international guidelines
- BRecommended in selected cases
- CStudy data contradict each other
- DBenefit not proven, no documented harm
- FDocumented harm, or risk without benefit