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pulm.idiopathic_pulmonary_fibrosis.v1PRODUCTION
pulm.idiopathic_pulmonary_fibrosis.v1

Idiopathic pulmonary fibrosis (IPF)

pulmonologychronicsubacuteadultgeriatric
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12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Adult chronic progressive fibrosing ILD — confirm UIP pattern on HRCT and adjudicate via multidisciplinary discussion (MDD: pulm + thoracic radiology + pathology) per ATS/ERS/JRS/ALAT 2022; scope excludes secondary fibrosis (CTD/drug/sarcoid), NSIP, HP

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IPF confirmed at MDD

Patient inputs (16)

IPF typically >60; GAP age points; transplant candidacy (ATS/ERS 2022)

GAP "G" term — male sex confers worse prognosis (Ley 2012 PMID 22586007)

Resting + ambulatory hypoxemia drives O2 prescription; LTOT trigger SpO2 ≤88% (ATS/ERS 2022)

Asbestos / silica / mold / bird / hot-tub exposure for asbestosis & HP (ATS 2018 dx)

Drug-induced ILD screen (amiodarone, MTX, nitrofurantoin, ICI, T-DXd); nintedanib + anticoagulant/CYP interactions (ATS/ERS 2022)

Exclude CTD-ILD before labelling IPF — ANA/RF/CCP/Scl-70/Jo-1/PL-7/PL-12/MDA5/Ro52 (ATS 2018 dx)

Anemia worsens dyspnea; pre-antifibrotic baseline; nintedanib bleeding risk (ATS/ERS 2022)

Pirfenidone / nintedanib hepatotoxicity baseline + serial LFT (DailyMed labels)

UIP-pattern HRCT is the central diagnostic axis; definite-UIP obviates biopsy (ATS 2018 dx)

FVC + DLCO baseline + GAP physiology; FVC trend drives progression/transplant (ATS/ERS 2022)

Functional capacity + exertional desaturation; nadir SpO2 prognostic (ATS/ERS 2022)

Screen Group-3 PH-ILD (TRV, RV function) — poor-prognosis modifier (ATS/ERS 2022)

Surgical/cryobiopsy ONLY when HRCT not definite UIP and MDD requires histology (ATS 2018 dx)

GERD highly prevalent; anti-reflux is a CONDITIONAL ATS/ERS 2022 recommendation

IgG precipitins for chronic fibrotic HP differential (ATS/JRS/ALAT 2020 HP)

HF / Group-2 PH differential vs Group-3 PH-ILD (ATS/ERS 2022)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (8)

8 need judgement
  • informationallife_threateningacute_exacerbation_ipf
    AE-IPF (Collard 2016 PMID 27299520) — acute worsening dyspnea <30 d + new bilateral GGO/consolidation NOT fully explained by HF, fluid overload, PE or infection
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateningdifferential_ae_ipf_vs_mimics
    Acute deterioration on background IPF — AE-IPF vs mimic? §5.5.2 AE-IPF differential (Collard 2016 PMID 27299520): infection (viral PCR + bacterial + PCP if immunosuppressed) vs PE (CTPA — D-dimer NON-diagnostic in IPF) vs cardiogenic edema (BNP↑↑, echo LV dysfunction) vs aspiration (witnessed/risk factors) vs drug-induced ILD (recent culprit drug) vs idiopathic AE-IPF (diagnosis of exclusion)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveregap_stage_3_or_rapid_progression
    GAP Stage III (8–12 pts) OR rapid progression (FVC ≥10% relative decline in 6 mo OR DLCO ≥15% decline) — 1-yr mortality ~39%
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveresevere_hypoxemia_resting
    Resting SpO2 ≤88% on room air
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverelegacy_triple_therapy_present
    Patient found on prednisone + azathioprine + N-acetylcysteine triple therapy for IPF
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatepulmonary_hypertension_ild
    Echo TRV elevated / RV dysfunction in IPF (Group-3 PH-ILD)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateantifibrotic_hepatotoxicity
    AST/ALT >3× ULN (or >5× ULN, or bilirubin >2× ULN) on pirfenidone or nintedanib
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatedifferential_uip_vs_ild_mimics
    Fibrotic ILD — is it IPF/UIP or a mimic? §5.5.2 pivots: definite-UIP HRCT (basal/subpleural reticulation + honeycombing + traction bronchiectasis, no inconsistent features) has ~90-100% specificity for histologic UIP → biopsy can be omitted (ATS 2018 PMID 30168753). Discriminators carry test characteristics — NSIP (ground-glass-predominant, no honeycomb, younger, steroid-responsive); chronic fibrotic HP (antigen Hx + 3-density mosaic air-trapping sign + BAL lymphocytosis >30% + IgG precipitins); CTD-ILD (female + ANA/RF/CCP/Scl-70/Jo-1/MDA5/Ro52 + extrathoracic features); drug-induced ILD (temporal exposure — amiodarone/MTX/nitrofurantoin/ICI/T-DXd; reversible if stopped); sarcoidosis (upper-lobe perilymphatic nodules + hilar adenopathy); CPFE (upper-lobe emphysema + lower-lobe fibrosis, preserved volumes, ↓↓DLCO, disproportionate PH); asbestosis (asbestos Hx + pleural plaques)
    Trigger could not be auto-evaluated — needs clinician judgement.

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RISK_STRATIFICATIONrequiredDrives severity classification
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Recommended regimen

IPF antifibrotic + supportive — chronic progressive, ATS/ERS/JRS/ALAT 2022
axis: ipf_antifibrotic
Selected axis "IPF antifibrotic + supportive — chronic progressive, ATS/ERS/JRS/ALAT 2022" by default fallback (first axis)
  • pirfenidone
    first line
    antifibrotic_pleiotropic
    267 mg PO TID wk1, 534 mg TID wk2, then 801 mg TID maintenance • PO • TID WITH FOOD (max: 2403 mg/day)
    triggers: confirmed_IPF, GI_intolerant_to_nintedanib, on_anticoagulation_or_high_bleed_risk
    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
    rxcui 1592254
  • nintedanib
    first line
    antifibrotic_intracellular_TKI
    150 mg PO BID (consider 100 mg BID if hepatic impairment / poor tolerance) • PO • BID WITH FOOD (max: 300 mg/day)
    triggers: confirmed_IPF, pirfenidone_intolerant_or_photosensitivity_concern, non_IPF_progressive_fibrosing_ILD_or_SSc_ILD
    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
    rxcui 1592737
  • oxygen
    comorbidity specific
    oxygen
    titrate to resting SpO2 ≥90%; ambulatory O2 for exertional desaturation • inhaled • continuous if resting SpO2 ≤88%
    triggers: resting_SpO2_le_88, exertional_desaturation_lt_88_on_6MWT
    ATS/ERS 2022 — LTOT for resting hypoxemia (SpO2 ≤88%); ambulatory O2 for isolated exertional desaturation (symptom/QoL benefit)
    rxcui 7806
  • pulmonary rehabilitation
    comorbidity specific
    rehabilitation
    structured exercise + education program • n/a • program course; maintenance thereafter
    triggers: symptomatic_IPF, exertional_limitation
    ATS/ERS 2022 — conditional recommendation; improves 6MWD, dyspnea, QoL
  • lung transplant referral
    first line
    definitive_therapy
    EARLY referral at diagnosis if no absolute contraindication • n/a • one-time referral + ongoing co-management
    triggers: confirmed_IPF_without_absolute_contraindication, FVC_decline_ge_10pct_6mo, DLCO_lt_40, resting_or_exertional_hypoxemia, GAP_stage_II_III
    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
    comorbidity specific
    PPI
    20–40 mg • PO • once daily
    triggers: symptomatic_GERD
    Anti-reflux is CONDITIONAL (low-quality evidence; 2022 update softened the 2015 recommendation) — treat symptomatic GERD; routine anti-acid for asymptomatic IPF NOT recommended
    rxcui 7646
  • esomeprazole
    contraindication substitute
    PPI
    20–40 mg • PO • once daily
    triggers: symptomatic_GERD, omeprazole_CYP_interaction_with_other_meds
    PPI alternative when omeprazole CYP2C19 interactions are problematic; same CONDITIONAL anti-reflux rationale
    rxcui 283742
  • methylprednisolone
    rescue
    systemic_corticosteroid
    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
    triggers: AE_IPF_after_infection_PE_HF_excluded
    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
    rxcui 6902
  • prednisone
    rescue
    systemic_corticosteroid
    0.5–1 mg/kg/day PO • PO • taper over weeks
    triggers: AE_IPF_oral_route, mild_AE_IPF
    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)
    rxcui 8640
  • nintedanib 100 mg BID (hepatic impairment — Child-Pugh A; AVOID in Child-Pugh B/C)
    contraindication substitute
    antifibrotic_intracellular_TKI
    100 mg PO BID (reduced) • PO • BID with food
    triggers: Child_Pugh_A_hepatic_impairment
    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
    rxcui 1592737
  • pirfenidone (anticoagulated patient — preferred over nintedanib)
    comorbidity specific
    antifibrotic_pleiotropic
    801 mg TID maintenance (standard titration) • PO • TID with food
    triggers: therapeutic_anticoagulation, high_bleeding_risk, recent_major_bleed
    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
    rxcui 1592254
  • antifibrotic peri-transplant management
    comorbidity specific
    transplant_bridge
    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
    triggers: listed_for_transplant, peri_operative_window
    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)
    rescue
    critical_care_supportive
    O2 to SpO2 ≥90%, HFNC/NIV trial, broad infection workup + empiric coverage, VTE assessment • n/a • continuous during AE-IPF
    triggers: 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)
    comorbidity specific
    antifibrotic_pleiotropic
    267 mg TID slow titration; individualise • PO • TID with food
    triggers: age_ge_75, CPFE_phenotype, multimorbidity_polypharmacy
    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)
    rxcui 1592254

outpatient playbook — drug actions (3)

  1. 1. pirfenidone OR nintedanib
    pirfenidone 267→801 mg TID OR nintedanib 150 mg BID (100 mg BID if Child-Pugh A) • PO • TID / BID with food
    trigger: Confirmed IPF at MDD (any FVC)
    Slow FVC decline — ASCEND PMID 24836312 / INPULSIS PMID 24836310; choose by comorbidity (anticoagulation→pirfenidone; photosensitivity→nintedanib)
  2. 2. ambulatory / long-term oxygen
    titrate SpO2 ≥90% • inhaled • continuous if resting SpO2 ≤88%
    trigger: Resting or exertional hypoxemia
    ATS/ERS 2022 — LTOT resting ≤88%; ambulatory O2 for exertional desaturation
  3. 3. PPI (symptomatic GERD only)
    omeprazole 20–40 mg • PO • daily
    trigger: Symptomatic GERD
    ATS/ERS 2022 CONDITIONAL anti-reflux — not routine in asymptomatic IPF

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Progressive dyspnea on exertion (>3–6 mo) (ATS/ERS 2022); Persistent dry cough (ATS/ERS 2022); Bibasilar Velcro inspiratory crackles ± digital clubbing (ATS 2018 dx).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Idiopathic pulmonary fibrosis (IPF)** (pulm.idiopathic_pulmonary_fibrosis.v1).
Phenotype framing: §5.5.2 differential-as-data — IPF/UIP vs NSIP (younger, ground-glass-predominant, no honeycomb, steroid-responsive) vs chronic fibrotic HP (antigen Hx, mosaic air-trapping 3-density sign, BAL lymphocytosis >30%, IgG precipitins → reconsider HP) vs CTD-ILD (female, +ANA/RF/CCP/Scl-70/Jo-1/MDA5, extrathoracic features → rheum MDD) vs drug-induced ILD (temporal drug exposure — amiodarone/MTX/nitrofurantoin/ICI/T-DXd; reversible if stopped) vs sarcoidosis (upper-lobe, perilymphatic nodules, hilar adenopathy → pulm.sarcoidosis.v1) vs CPFE (upper-lobe emphysema + lower-lobe fibrosis, preserved spirometric volumes, severely ↓DLCO, disproportionate PH → pulm.copd.core.v1 overlap) vs asbestosis (asbestos Hx + pleural plaques). Definite-UIP HRCT has high specificity (~90-100%) for histologic UIP → biopsy can be omitted (ATS 2018 PMID 30168753)
Scope: Adult chronic progressive fibrosing ILD — confirm UIP pattern on HRCT and adjudicate via multidisciplinary discussion (MDD: pulm + thoracic radiology + pathology) per ATS/ERS/JRS/ALAT 2022; scope excludes secondary fibrosis (CTD/drug/sarcoid), NSIP, HP

No severity triggers fired against current inputs.

Plan

Regimen axis: **IPF antifibrotic + supportive — chronic progressive, ATS/ERS/JRS/ALAT 2022**.
1. pirfenidone 267 mg PO TID wk1, 534 mg TID wk2, then 801 mg TID maintenance PO TID WITH FOOD (antifibrotic_pleiotropic, first line) — 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
2. nintedanib 150 mg PO BID (consider 100 mg BID if hepatic impairment / poor tolerance) PO BID WITH FOOD (antifibrotic_intracellular_TKI, first line) — 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
3. oxygen titrate to resting SpO2 ≥90%; ambulatory O2 for exertional desaturation inhaled continuous if resting SpO2 ≤88% (oxygen, comorbidity specific) — ATS/ERS 2022 — LTOT for resting hypoxemia (SpO2 ≤88%); ambulatory O2 for isolated exertional desaturation (symptom/QoL benefit)
4. pulmonary rehabilitation structured exercise + education program n/a program course; maintenance thereafter (rehabilitation, comorbidity specific) — ATS/ERS 2022 — conditional recommendation; improves 6MWD, dyspnea, QoL
5. lung transplant referral EARLY referral at diagnosis if no absolute contraindication n/a one-time referral + ongoing co-management (definitive_therapy, first line) — Lung transplant is the ONLY disease-modifying option; IPF has worst waitlist mortality among ILDs — refer early (ISHLT candidate-selection principles; ATS/ERS 2022)
6. omeprazole 20–40 mg PO once daily (PPI, comorbidity specific) — Anti-reflux is CONDITIONAL (low-quality evidence; 2022 update softened the 2015 recommendation) — treat symptomatic GERD; routine anti-acid for asymptomatic IPF NOT recommended
7. esomeprazole 20–40 mg PO once daily (PPI, contraindication substitute) — PPI alternative when omeprazole CYP2C19 interactions are problematic; same CONDITIONAL anti-reflux rationale
8. 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 (systemic_corticosteroid, rescue) — 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
9. prednisone 0.5–1 mg/kg/day PO PO taper over weeks (systemic_corticosteroid, rescue) — 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)
10. nintedanib 100 mg BID (hepatic impairment — Child-Pugh A; AVOID in Child-Pugh B/C) 100 mg PO BID (reduced) PO BID with food (antifibrotic_intracellular_TKI, contraindication substitute) — 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
11. pirfenidone (anticoagulated patient — preferred over nintedanib) 801 mg TID maintenance (standard titration) PO TID with food (antifibrotic_pleiotropic, comorbidity specific) — 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
12. 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 (transplant_bridge, comorbidity specific) — 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
13. 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 (critical_care_supportive, rescue) — 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)
14. pirfenidone (elderly / comorbid CPFE — start best-tolerated, intensify monitoring) 267 mg TID slow titration; individualise PO TID with food (antifibrotic_pleiotropic, comorbidity specific) — 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)

Setting playbook (outpatient) — Confirm IPF at MDD, stage with GAP, initiate + monitor antifibrotic, oxygen, pulmonary rehab, EARLY transplant referral, comorbidity sweep, palliative integration (ATS/ERS 2022)
15. pirfenidone OR nintedanib pirfenidone 267→801 mg TID OR nintedanib 150 mg BID (100 mg BID if Child-Pugh A) PO TID / BID with food — Confirmed IPF at MDD (any FVC) (Slow FVC decline — ASCEND PMID 24836312 / INPULSIS PMID 24836310; choose by comorbidity (anticoagulation→pirfenidone; photosensitivity→nintedanib))
16. ambulatory / long-term oxygen titrate SpO2 ≥90% inhaled continuous if resting SpO2 ≤88% — Resting or exertional hypoxemia (ATS/ERS 2022 — LTOT resting ≤88%; ambulatory O2 for exertional desaturation)
17. PPI (symptomatic GERD only) omeprazole 20–40 mg PO daily — Symptomatic GERD (ATS/ERS 2022 CONDITIONAL anti-reflux — not routine in asymptomatic IPF)

Non-pharmacologic actions:
- Pulmonary rehabilitation (ATS/ERS 2022 conditional)
- EARLY lung transplant referral at diagnosis if no absolute contraindication (ISHLT principles)
- Annual vaccinations (ATS/ERS 2022)
- Smoking cessation (ATS/ERS 2022)
- Palliative care + advance care planning introduced early (ATS/ERS 2022)
- Lung-cancer surveillance — IPF confers elevated risk (ATS/ERS 2022)

AVOID / contraindication checks:
- Pirfenidone photosensitivity take with food sun protection (DailyMed pirfenidone label)
- Pirfenidone hepatotoxicity LFT baseline then monthly x6 then q3m dose reduce or stop if AST ALT gt 3x or bilirubin (DailyMed pirfenidone label; ATS/ERS 2022)
- Nintedanib hepatotoxicity LFT baseline then monthly x3 then periodic (DailyMed nintedanib label)
- Nintedanib bleeding and arterial thromboembolic risk caution on full anticoagulation (DailyMed nintedanib label)
- Nintedanib CYP3A4 Pgp substrate avoid strong inducers inhibitors (DailyMed nintedanib label)
- Nintedanib GI perforation and diarrhoea loperamide then dose reduce 150 to 100 BID (DailyMed nintedanib label)
- Nintedanib not recommended Child Pugh B C reduce to 100 BID Child Pugh A (DailyMed nintedanib label)
- NO triple therapy prednisone azathioprine NAC HARMFUL stop legacy triple (PANTHER IPF Raghu NEJM 2012 PMID 22607134)
- AE IPF high dose corticosteroid weak no RCT evidence individualise treat precipitant (Collard 2016 PMID 27299520; ATS/ERS 2022 conditional against)
- Anti reflux conditional only treat symptomatic GERD not routine asymptomatic (ATS/ERS 2022 update)
- Avoid routine invasive ventilation AE IPF unless transplant bridge align goals of care (ATS/ERS 2022)
- NAC monotherapy not effective in IPF (PANTHER IPF PMID 22607134)

Monitoring

Regimen monitoring:
- LFT baseline then monthly x6 then q3m pirfenidone (DailyMed; ATS/ERS 2022)
- LFT baseline then monthly x3 then periodic nintedanib (DailyMed nintedanib label)
- CBC periodic and bleeding review on nintedanib (DailyMed nintedanib label)
- weight and GI tolerance on nintedanib (DailyMed nintedanib label)
- photosensitivity and rash counselling on pirfenidone (DailyMed pirfenidone label)
- FVC q3-6m progression trigger ge 10pct relative decline (ATS/ERS 2022; ASCEND PMID 24836312)
- DLCO q6-12m (ATS/ERS 2022)
- 6MWT q6m with continuous SpO2 for exertional desaturation (ATS/ERS 2022)
- HRCT annually or per change (ATS/ERS 2022)
- GAP stage recompute for transplant timing (Ley 2012 PMID 22586007)
- screen Group3 PH ILD and lung cancer surveillance annually (ATS/ERS 2022)

Setting (outpatient) monitoring:
- FVC q3–6 mo, DLCO q6–12 mo, 6MWT q6 mo, HRCT annually (ATS/ERS 2022)
- LFT monthly ×6 then q3 mo (pirfenidone) / monthly ×3 then periodic (nintedanib) (DailyMed)
- GAP recompute for transplant timing (Ley 2012 PMID 22586007)

Follow-up plan: 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)
- Close-out criterion: Follow-up + transplant + comorbidity + palliative loop booked

Monitoring phase: FVC q3–6 mo + DLCO q6–12 mo (progression trend drives transplant), 6MWT q6 mo, HRCT annually or per change. Antifibrotic safety: LFT monthly ×6 then q3 mo (both drugs), CBC, weight (nintedanib GI), photosensitivity counselling (pirfenidone). Track ≥10% relative FVC decline = progression trigger (ATS/ERS 2022)

Disposition

Current setting: outpatient — Confirm IPF at MDD, stage with GAP, initiate + monitor antifibrotic, oxygen, pulmonary rehab, EARLY transplant referral, comorbidity sweep, palliative integration (ATS/ERS 2022)

Disposition criteria:
- Continue surveillance + antifibrotic + supportive bundle if stable (ATS/ERS 2022)
- Expedite transplant clinic if GAP Stage II/III or rapid progression

Escalation triggers (move to higher acuity):
- Acute exacerbation (acute dyspnea + new GGO) → ED (Collard 2016 PMID 27299520)
- Resting hypoxemia SpO2 ≤88% → LTOT (ATS/ERS 2022)
- FVC ≥10% relative decline in 6 mo or DLCO ≤35% → expedite transplant evaluation + palliative (Ley 2012)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] AE-IPF (Collard 2016 PMID 27299520) — acute worsening dyspnea <30 d + new bilateral GGO/consolidation NOT fully explained by HF, fluid overload, PE or infection
- [LIFE_THREATENING] Acute deterioration on background IPF — AE-IPF vs mimic? §5.5.2 AE-IPF differential (Collard 2016 PMID 27299520): infection (viral PCR + bacterial + PCP if immunosuppressed) vs PE (CTPA — D-dimer NON-diagnostic in IPF) vs cardiogenic edema (BNP↑↑, echo LV dysfunction) vs aspiration (witnessed/risk factors) vs drug-induced ILD (recent culprit drug) vs idiopathic AE-IPF (diagnosis of exclusion)
- [SEVERE] GAP Stage III (8–12 pts) OR rapid progression (FVC ≥10% relative decline in 6 mo OR DLCO ≥15% decline) — 1-yr mortality ~39%

Citations

- 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). [PMID:35486072](https://pubmed.ncbi.nlm.nih.gov/35486072/)
- Cited evidence (PMID 30168753) [PMID:30168753](https://pubmed.ncbi.nlm.nih.gov/30168753/)
- Cited evidence (PMID 24836312) [PMID:24836312](https://pubmed.ncbi.nlm.nih.gov/24836312/)
- Cited evidence (PMID 21571362) [PMID:21571362](https://pubmed.ncbi.nlm.nih.gov/21571362/)
- Cited evidence (PMID 24836310) [PMID:24836310](https://pubmed.ncbi.nlm.nih.gov/24836310/)

Last reconciled with current guidelines: 2026-05-26.
References
  • 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). — PMID:35486072
  • Cited evidence (PMID 30168753) — PMID:30168753
  • Cited evidence (PMID 24836312) — PMID:24836312
  • Cited evidence (PMID 21571362) — PMID:21571362
  • Cited evidence (PMID 24836310) — PMID:24836310