This handout is for idiopathic pulmonary fibrosis (ipf). Your care team identified this based on: progressive dyspnea on exertion (>3–6 mo) (ats/ers 2022).
Other reasons your team may use this plan: persistent dry cough (ats/ers 2022); bibasilar velcro inspiratory crackles ± digital clubbing (ats 2018 dx); hrct uip pattern (basal, subpleural, reticulation, honeycombing, traction bronchiectasis) (ats 2018 dx).
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| pirfenidone | 267 mg PO TID wk1, 534 mg TID wk2, then 801 mg TID maintenance | PO | TID WITH FOOD | ASCEND (King NEJM 2014 PMID 24836312): ≥10% FVC decline-or-death relative reduction ~47.9% at 52 wk; CAPACITY pooled (Noble Lancet 2011 PMID 21571362) confirmatory. Prefer when nintedanib bleeding/anticoagulant risk dominates. Photosensitivity + GI + hepatotoxicity — take with food, sun protection; reduce to 534 then 267 mg TID for ADR |
| nintedanib | 150 mg PO BID (consider 100 mg BID if hepatic impairment / poor tolerance) | PO | BID WITH FOOD | INPULSIS-1/2 (Richeldi NEJM 2014 PMID 24836310): annual FVC −114.7 vs −239.9 mL (Δ ~125 mL/yr); also slows non-IPF PF-ILD (INBUILD PMID 31566307: −80.8 vs −187.8 mL/yr) and SSc-ILD (SENSCIS PMID 31112379). Diarrhoea most common ADR (loperamide + dose-reduce 150→100 mg BID); hepatotoxicity; bleeding + arterial thromboembolic risk — caution on full anticoagulation; CYP3A4/P-gp substrate |
| oxygen | titrate to resting SpO2 ≥90%; ambulatory O2 for exertional desaturation | inhaled | continuous if resting SpO2 ≤88% | ATS/ERS 2022 — LTOT for resting hypoxemia (SpO2 ≤88%); ambulatory O2 for isolated exertional desaturation (symptom/QoL benefit) |
| pulmonary rehabilitation | structured exercise + education program | n/a | program course; maintenance thereafter | ATS/ERS 2022 — conditional recommendation; improves 6MWD, dyspnea, QoL |
| lung transplant referral | EARLY referral at diagnosis if no absolute contraindication | n/a | one-time referral + ongoing co-management | Lung transplant is the ONLY disease-modifying option; IPF has worst waitlist mortality among ILDs — refer early (ISHLT candidate-selection principles; ATS/ERS 2022) |
| omeprazole | 20–40 mg | PO | once daily | Anti-reflux is CONDITIONAL (low-quality evidence; 2022 update softened the 2015 recommendation) — treat symptomatic GERD; routine anti-acid for asymptomatic IPF NOT recommended |
| esomeprazole | 20–40 mg | PO | once daily | PPI alternative when omeprazole CYP2C19 interactions are problematic; same CONDITIONAL anti-reflux rationale |
| methylprednisolone | 500–1000 mg IV daily ×3 (pulse), then taper; OR prednisone 0.5–1 mg/kg/day | IV/PO | pulse ×3 then taper over weeks | AE-IPF in-hospital mortality 50-60% (Collard 2016 PMID 27299520). High-dose corticosteroid is common practice but WEAK/no-RCT evidence (conditional-against in 2022 guideline; individualise + treat precipitant); continue antifibrotic if tolerating PO |
| prednisone | 0.5–1 mg/kg/day PO | PO | taper over weeks | Oral corticosteroid option for AE-IPF when IV pulse not used. NEVER as part of triple therapy (PANTHER PMID 22607134 — pred+azathioprine+NAC increased death & hospitalisation; STOP if patient on legacy triple) |
| nintedanib 100 mg BID (hepatic impairment — Child-Pugh A; AVOID in Child-Pugh B/C) | 100 mg PO BID (reduced) | PO | BID with food | Special-pop: hepatic impairment branch — nintedanib Child-Pugh A reduce to 100 mg BID; NOT recommended Child-Pugh B/C (DailyMed label). Pirfenidone also requires caution / dose-reduction in moderate hepatic impairment and is contraindicated in severe — prefer best-tolerated agent with intensified LFT |
| pirfenidone (anticoagulated patient — preferred over nintedanib) | 801 mg TID maintenance (standard titration) | PO | TID with food | Special-pop: anticoagulated branch — nintedanib carries bleeding + arterial thromboembolic signal and VEGFR inhibition; in patients on full-dose anticoagulation or high bleed risk, prefer PIRFENIDONE. If nintedanib unavoidable, co-manage anticoagulation intensity and monitor closely |
| antifibrotic peri-transplant management | continue antifibrotic to bridge waitlist; coordinate hold timing with transplant surgery (wound-healing/bleeding considerations — nintedanib anti-angiogenic) | n/a | until transplant per center protocol | Special-pop: peri-transplant branch — antifibrotics bridge progression on the waitlist; nintedanib VEGFR inhibition raises perioperative wound-healing/bleeding concern → hold timing is center-specific; do not abruptly stop without transplant-team coordination |
| AE-IPF supportive bundle (HFNC/NIV, treat precipitant, palliative-aligned ICU decision) | O2 to SpO2 ≥90%, HFNC/NIV trial, broad infection workup + empiric coverage, VTE assessment | n/a | continuous during AE-IPF | Special-pop: AE-IPF branch — invasive ventilation has very high mortality and should generally be reserved for transplant candidates as a bridge; align ICU/ECMO escalation with goals of care and palliative input (Collard 2016 PMID 27299520) |
| pirfenidone (elderly / comorbid CPFE — start best-tolerated, intensify monitoring) | 267 mg TID slow titration; individualise | PO | TID with food | Special-pop: elderly / CPFE branch — CPFE (emphysema + lower-lobe fibrosis) has preserved spirometric volumes but severe ↓DLCO and disproportionate PH; antifibrotic still indicated for the fibrotic component. In frail elderly use slow titration, the best-tolerated agent, and tighter LFT/ADR surveillance; screen Group-3 PH (route pulm.pulmonary_htn_group2_to_5.v1) |
Plan: IPF antifibrotic + supportive — chronic progressive, ATS/ERS/JRS/ALAT 2022
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
ILD clinic q3–6 mo, transplant clinic co-management, comorbidity sweep (Group-3 PH, CPFE, GERD, OSA, lung-cancer surveillance — IPF raises lung-ca risk, depression), pulmonary rehab continuity, palliative care + advance care planning (ATS/ERS 2022)
Guideline: ATS/ERS/JRS/ALAT 2022 IPF (update) + Progressive Pulmonary Fibrosis Guideline (PMID 35486072); ATS/ERS/JRS/ALAT 2018 IPF Diagnosis Guideline (PMID 30168753). Current as of 2026-05-18 — no 2024/25 ATS/ERS/JRS/ALAT IPF replacement (WebSearch-confirmed).