Idiopathic Pulmonary Fibrosis Medical Slides

Present the 2022 guideline algorithm: Definite UIP pattern (honeycombing + basal-predominant distribution ± traction bronchiectasis/bronchiolectasis) in the right clinical context = IPF diagnosis without biopsy (PPV >90%). Probable UIP (basal reticulation + traction bronchiectasis without honeycombing) — conditional recommendation for IPF diagnosis without biopsy. Indeterminate/alternative pattern — consider surgical lung biopsy, transbronchial cryobiopsy (2019 COLDICE trial), or MDD discussion.

Present head-to-head comparison: Pirfenidone (ASCEND, 2014): reduced proportion of patients with ≥10% FVC decline from 17% to 10%, also reduced 6MWD decline. Nintedanib (INPULSIS 1&2, 2014): reduced annual FVC decline by approximately 125 mL/year. Neither demonstrated mortality benefit in individual trials. No head-to-head RCT exists. Side effects: pirfenidone — photosensitivity, nausea, rash; nintedanib — diarrhea (62%), hepatotoxicity. Both should be started early after diagnosis.

Present the 2021 ISHLT referral criteria for IPF: refer at diagnosis regardless of lung function (given unpredictable trajectory). Urgent listing criteria: FVC <80% predicted, DLCO <40%, ≥5% FVC decline in 6 months, 6MWD <250 m or >50 m decline in 6 months, GAP Index stage II-III, pulmonary hypertension on RHC, hospitalization for respiratory decline. Median wait time 2-3 years — early referral is critical. Post-transplant median survival approximately 5 years for IPF.