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Pulmonary Medicine›Condition·Updated Jun 27, 2026·v1

Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is a progressive fibrotic interstitial lung disease with a UIP pattern on HRCT/histopathology, median survival 3-5 years, and strong genetic predisposition (MUC5B, telomerase genes). Diagnosis requires multidisciplinary discussion. Management centers on antifibrotic therapy (nintedanib or pirfenidone), oxygen therapy, pulmonary rehabilitation, and early lung transplant referral. Acute exacerbations carry >50% in-hospital mortality and are treated with high-dose corticosteroids; immunosuppressants are contraindicated.

High Evidence555 references·3,489 words·14 min read·v1
Idiopathic Pulmonary FibrosisInterstitial Lung DiseasePulmonary MedicineAntifibrotic TherapyUsual Interstitial PneumoniaUIPNintedanibPirfenidone

Quick Reference

RxDrug of choiceNintedanib 150 mg PO BID or pirfenidone 2403 mg/day (801 mg TID) - both first-line, no significant difference in efficacy.
AltAlternativesNerandomilast 18 mg BID (PDE4B inhibitor) or inhaled treprostinil 12 breaths QID for progressive disease despite first-line therapy.
⊘AvoidImmunosuppressants (prednisone+azathioprine+NAC triple therapy - PANTHER trial showed increased mortality); cyclophosphamide for AE-IPF (EXAFIP trial showed no benefit); anticoagulation for AE-IPF (ACE-IPF trial showed harm).
DxTest of choiceHigh-resolution CT (HRCT) with definite UIP pattern (reticulation, honeycombing, traction bronchiectasis, subpleural/basal predominance) - sufficient for diagnosis without biopsy in appropriate clinical context.
ScKey scoreGAP index (Gender, Age, FVC, DLCO) - stratifies 1-year mortality: stage I 6%, stage II 16%, stage III 39%.
→When to referRefer for lung transplantation evaluation when disease progresses despite antifibrotic therapy, DLCO <39% predicted, 6-minute walk distance <250 m, or after an acute exacerbation.
IPF is a progressive fibrotic lung disease with median survival 3-5 years; early diagnosis via HRCT and multidisciplinary discussion, prompt initiation of nintedanib or pirfenidone, and timely referral for lung transplantation are essential to improve outcomes.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial lung disease of unknown cause, defined by a usual interstitial pneumonia (UIP) pattern on HRCT or histopathology after excluding other etiologies. It is the most common and most lethal of the idiopathic interstitial pneumonias, with a median survival of 3-5 years from diagnosis. This reference summarizes key clinical facts, drug doses, critical thresholds, and management algorithms for bedside use.

Overview and Recommendations

Background

  • •IPF is the most common and lethal idiopathic interstitial pneumonia, with a median survival of 3-5 years from diagnosis and a 1-year mortality of 39% at GAP stage III. It accounts for ~50% of all interstitial lung disease cases and affects 14-43 per 100,000 persons in the US, with incidence rising sharply after age 65.
  • •The disease is defined by a UIP pattern on HRCT (reticulation, honeycombing, traction bronchiectasis, subpleural/basal predominance) or histopathology (patchy fibrosis, fibroblastic foci, architectural distortion) after excluding other causes. Multidisciplinary discussion among pulmonologists, radiologists, and pathologists is the gold standard for diagnosis.
  • •The strongest genetic risk factor is the MUC5B promoter polymorphism (rs35705950), present in ~30-40% of IPF patients versus ~10% of controls, conferring a 4- to 8-fold increased risk. Rare variants in telomerase genes (TERT, TERC, PARN, RTEL1) cause familial pulmonary fibrosis and accelerate disease onset.
  • •Pathogenesis involves repetitive microinjury to a genetically vulnerable alveolar epithelium, triggering dysregulated wound healing driven by TGF-β (master profibrotic cytokine), WNT/β-catenin signaling, and metabolic reprogramming toward aerobic glycolysis. Cellular senescence, mitochondrial dysfunction, and a profibrotic SPP1/MERTK macrophage population perpetuate fibrosis.
  • •IPF is a restrictive lung disease with reduced FVC and DLCO. The annual rate of FVC decline in untreated patients is 150-200 mL/year. Acute exacerbations (AE-IPF) occur at an annual incidence of 5-15% and carry an in-hospital mortality >50%, defined by acute respiratory worsening with new bilateral ground-glass opacities on HRCT without an identifiable trigger.

Evaluation

  • •Suspect IPF in any patient over 60 years with insidious exertional dyspnea, dry cough, and bilateral basilar 'Velcro-like' inspiratory crackles that persist unchanged by cough or position. Clubbing is present in 25-50% and increases specificity for IPF over other fibrotic ILDs.
  • •Ask about smoking history (ever-smokers have 2- to 3-fold increased risk), occupational exposures (metal dust, wood dust, silica, agriculture), family history of pulmonary fibrosis, and symptoms of connective tissue disease (arthralgias, Raynaud phenomenon, rash). Diagnostic delay averages 1-2 years because symptoms are often attributed to aging or deconditioning.
  • •Examine for signs of pulmonary hypertension (loud P2, right ventricular heave, elevated JVP, peripheral edema) in advanced disease. Assess for digital clubbing and auscultate for crackles. Measure oxygen saturation at rest and during a 6-minute walk test.
  • •Order high-resolution CT (HRCT) as the cornerstone of noninvasive diagnosis. A definite UIP pattern (reticulation with honeycombing, traction bronchiectasis, subpleural/basal predominance, minimal ground-glass) in a patient >60 years with typical symptoms is sufficient to diagnose IPF without lung biopsy.
  • •If HRCT shows probable UIP (reticulation with traction bronchiectasis but no honeycombing), indeterminate, or alternative patterns, proceed to multidisciplinary discussion and consider lung biopsy. Transbronchial lung cryobiopsy (TBLC) is preferred over surgical lung biopsy due to lower morbidity (pneumothorax ~9%, moderate bleeding ~5%).
  • •Perform pulmonary function tests: spirometry shows restrictive pattern (FVC <80% predicted, FEV1/FVC >0.70), plethysmography confirms reduced TLC, and DLCO is the most sensitive marker (often <60% predicted before FVC declines). A DLCO <40% predicted at presentation identifies highest risk for early mortality.
  • •Obtain a 6-minute walk test to quantify functional capacity; distance <250 m or desaturation to <88% carries independent prognostic weight. Serial PFTs every 3-6 months track progression: an absolute FVC decline ≥10% predicted or DLCO decline ≥15% predicted within 12 months defines progressive pulmonary fibrosis and triggers therapeutic escalation.
  • •Order serology for connective tissue disease (ANA, RF, anti-CCP, anti-Scl-70, anti-Ro52) and hypersensitivity pneumonitis panel (precipitins to avian, fungal antigens) to exclude alternative causes. Bronchoalveolar lavage (BAL) is used selectively when infection or HP is suspected; lymphocytosis >30% suggests HP.
  • •Assess GAP index (Gender, Age, FVC, DLCO) at baseline for risk stratification: stage I (0-3 points) 1-year mortality 6%, stage II (4-5) 16%, stage III (6-8) 39%. Consider adding telomere length measurement (TAP index) in younger patients or those with family history to improve discrimination.
  • •Also consider: combined pulmonary fibrosis and emphysema (CPFE) phenotype with preserved FVC but severely reduced DLCO and high risk of pulmonary hypertension; and familial pulmonary fibrosis when two or more first-degree relatives are affected, often with telomere-related gene mutations.

Management

  • •Initiate antifibrotic therapy at diagnosis for all patients with IPF regardless of baseline severity. First-line options are nintedanib 150 mg PO twice daily or pirfenidone 2403 mg/day (three 267-mg capsules three times daily). Choice is guided by tolerability, comorbidities, and patient preference; no significant difference in efficacy between agents.
  • •Start nintedanib at 150 mg BID; reduce to 100 mg BID if Child-Pugh A hepatic impairment; avoid if Child-Pugh B/C or eGFR <30 mL/min. Monitor LFTs monthly for 3 months then every 3 months. Manage diarrhea aggressively with loperamide, dietary modifications, and dose reduction if needed.
  • •Start pirfenidone at 267 mg TID (week 1), escalate to 534 mg TID (week 3), target 801 mg TID (2403 mg/day). Reduce dose if Child-Pugh A; avoid if Child-Pugh B. Monitor LFTs monthly for 6 months then every 3 months. Advise photosensitivity precautions (sun protection, clothing).
  • •Add emerging therapies for progressive disease despite first-line antifibrotic: nerandomilast 18 mg BID (PDE4B inhibitor) showed FVC benefit of 95.7 mL at 52 weeks and reduced acute exacerbations/hospitalization/death (HR 0.67) in FIBRONEER-IPF. Inhaled treprostinil 12 breaths QID improved FVC by 50.1 mL at 52 weeks in TETON-2 and reduced exacerbations (HR 0.64).
  • •Refer for lung transplantation evaluation early: when disease progresses despite antifibrotic therapy, DLCO falls below 39% predicted, 6-minute walk distance declines below 250 m, or after an acute exacerbation. Bilateral lung transplantation is preferred; median survival post-transplant is 5-7 years.
  • •Prescribe long-term oxygen therapy (LTOT) for resting hypoxemia (SpO2 ≤88% or PaO2 ≤55 mmHg) for at least 15 hours/day to maintain SpO2 ≥90%. LTOT reduces respiratory-related hospitalizations (rate ratio 0.79). For exertional desaturation, ambulatory oxygen improves exercise capacity but not survival.
  • •Enroll patients in pulmonary rehabilitation: an 8-week supervised program improves 6-minute walk distance by ~30 m and reduces dyspnea. Combine with home-based training to maintain benefits.
  • •Manage acute exacerbation of IPF (AE-IPF) with high-dose IV methylprednisolone 500-1000 mg daily for 3 days, then oral prednisolone taper over 4-6 weeks. Provide broad-spectrum antibiotics empirically while ruling out infection. Do NOT add cyclophosphamide (EXAFIP trial showed no benefit and trend to harm). Do NOT use anticoagulation.
  • •Provide supportive oxygen: maintain SpO2 ≥88% with nasal cannula, escalate to high-flow nasal cannula (40-60 L/min) if PaO2/FiO2 <200. Consider noninvasive ventilation for hypercapnia (PaCO2 >45 mmHg) or respiratory acidosis (pH <7.35). Intubate with lung-protective ventilation (tidal volume 6 mL/kg, plateau pressure ≤30 cmH2O) if refractory hypoxemia or NIV failure.
  • •Monitor for complications: pulmonary hypertension (screen with echo; treat with inhaled treprostinil if PH-ILD), lung cancer (5-10% incidence; multidisciplinary management), venous thromboembolism (prophylactic LMWH in hospitalized patients), and gastroesophageal reflux (PPI if symptomatic).
  • •Vaccinate against influenza, pneumococcus, and SARS-CoV-2. Avoid immunosuppressants (prednisone, azathioprine, mycophenolate) for IPF outside of acute exacerbation trials; the PANTHER trial showed increased mortality with triple therapy (prednisone+azathioprine+NAC).
  • •Assess frailty and goals of care regularly. The Clinical Frailty Scale (CFS) ≥5 predicts 1-year mortality after AE-IPF. Involve palliative care for symptom management (low-dose opioids for refractory dyspnea) and advance care planning.

Board Review — High Yield

  • •Usual interstitial pneumonia (UIP) pattern - Hallmark of IPF on HRCT: subpleural, basal predominant reticulation with honeycombing and traction bronchiectasis; minimal ground-glass opacity.
  • •MUC5B promoter polymorphism (rs35705950) - Strongest genetic risk factor (OR 4-8); present in 30-40% of IPF patients; associated with slower disease progression.
  • •GAP index - Prognostic tool using Gender, Age, FVC, DLCO; stage I (0-3 points) 1-year mortality 6%, stage II (4-5) 16%, stage III (6-8) 39%.
  • •Antifibrotic therapy - Nintedanib (150 mg BID) or pirfenidone (2403 mg/day) reduce FVC decline by ~50% and all-cause mortality by ~30% (NNT=22 over 12 months).
  • •PANTHER trial - Triple therapy (prednisone+azathioprine+NAC) increased mortality in IPF; contraindicated.
  • •Acute exacerbation of IPF (AE-IPF) - Acute respiratory worsening with new bilateral GGO on HRCT; in-hospital mortality >50%; treat with high-dose methylprednisolone; do NOT add cyclophosphamide.
  • •Transbronchial lung cryobiopsy (TBLC) - Preferred over surgical lung biopsy for tissue diagnosis; diagnostic yield ~80%, pneumothorax rate ~9%.
  • •Pulmonary hypertension in IPF - Prevalence 30-50% in advanced disease; treat with inhaled treprostinil (INCREASE trial); avoid bosentan and ambrisentan.
  • •Combined pulmonary fibrosis and emphysema (CPFE) - Phenotype with preserved FVC but severely reduced DLCO; high risk of pulmonary hypertension and lung cancer.
  • •Familial pulmonary fibrosis - Defined by ≥2 first-degree relatives with fibrosing ILD; often due to telomere-related gene mutations (TERT, TERC, PARN, RTEL1); younger age at onset.

Deep Dive — Evidence Details

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