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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
1. Definition, Classification and Nomenclature
- ▸IPF diagnosis requires UIP pattern on HRCT/histopathology after excluding other causes [1,2].
- ▸Median survival is 3-5 years; early antifibrotic therapy is critical [29,51].
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic of unknown cause, defined by a usual interstitial pneumonia (UIP) pattern on HRCT or histopathology after excluding other etiologies [1]A1c[2]A1c. The 2018 ATS/ERS/JRS/ALAT guideline classifies HRCT patterns into UIP, probable UIP, indeterminate for UIP, and alternative diagnosis [2]A1c. Probable UIP shows reticulation and traction bronchiectasis without honeycombing; it often requires lung biopsy for confirmation. The concept of progressive pulmonary fibrosis (PPF) describes worsening fibrosis in non-IPF fibrosing ILDs, defined by at least two of three criteria (symptoms, physiology, or radiologic progression) in the past year [17]D5[23]D5. IPF is a restrictive lung disease with reduced FVC and DLCO; median survival is 3 to 5 years from diagnosis [29]B2c[51]D5. Incidence in the US is 2.8-8.7 per 100,000 person-years, increasing with age [13]B2b[29]B2c[57]B2b. Acute exacerbation (AE-IPF) is an acute respiratory deterioration with new bilateral GGO/consolidation on a UIP background [15]D5. Familial pulmonary fibrosis (FPF) affects two or more first-degree relatives, often with telomere-related gene mutations [60]B2b[62]D5.
Pearl: IPF is defined by a UIP pattern on HRCT or histopathology after exclusion of other causes; its diagnosis requires multidisciplinary consensus, and the disease carries a median survival of 3 to 5 years, making early recognition and antifibrotic therapy critical [1]A1c[2]A1c[29]B2c.
| Pattern | HRCT Features | Histopathologic Correlate |
|---|---|---|
| UIP | Reticulation with honeycombing, traction bronchiectasis, subpleural and basal predominance; minimal ground-glass opacity | Dense fibrosis with architectural distortion, fibroblastic foci, honeycombing |
| Probable UIP | Reticulation with traction bronchiectasis, subpleural and basal predominance; no honeycombing | Same as UIP but less extensive or without honeycombing |
| Indeterminate for UIP | Reticulation with or without mild ground-glass opacity; distribution not typical of UIP | Fibrosis not meeting UIP criteria; may show features of other IIPs |
| Alternative diagnosis | Features suggesting another ILD (e.g., extensive ground-glass, nodules, cysts) | Histopathology of a different ILD (e.g., NSIP, hypersensitivity pneumonitis) |
2. Pathophysiology and Mechanism
- ▸MUC5B promoter variant (rs35705950) is the strongest genetic risk factor [115,136].
- ▸TGF-beta is the master profibrotic cytokine; nintedanib targets PDGF, FGF, and Src [94,95,101].
IPF pathogenesis begins with repetitive microinjury to a genetically vulnerable alveolar epithelium, triggering dysregulated wound healing [94]D5[103]D5. The strongest genetic risk factor is the MUC5B promoter polymorphism (rs35705950), which increases MUC5B expression, impairing mucociliary clearance [115]B3b[136]D5. Rare variants in telomerase genes (TERT, TERC) cause short telomeres, accelerating cellular senescence [116]C4[152]D5. Repeated injury leads to apoptosis and senescence of alveolar epithelial cells (AECs), which secrete a senescence-associated secretory phenotype (SASP) with inflammatory cytokines and matrix metalloproteinases [16]D5[87]C4. Transforming growth factor-beta (TGF-beta) is the master profibrotic cytokine, driving myofibroblast differentiation via SMAD-dependent pathways [94]D5[95]C4. WNT/beta-catenin signaling and metabolic reprogramming toward aerobic glycolysis are also key [86]C4[119]D5[138]D5. Nintedanib targets PDGF, FGF, and Src family kinases [94]D5[101]D5. Myofibroblasts deposit excessive extracellular matrix (ECM), with MMP-7 elevated and serving as a biomarker [91]D5[94]D5[147]B3b[150]D5. Vascular remodeling involves endothelial-to-mesenchymal transition (EndoMT) and pulmonary [90]C4[109]D5[140]D5. Immune dysregulation includes a profibrotic SPP1/MERTK-expressing macrophage population [93]B3b. Epithelial alarmins (TSLP, IL-25, IL-33) promote type 2 immune responses [141]D5.
Pearl: IPF pathogenesis is a multi-hit process integrating genetic susceptibility (MUC5B, telomerase), repetitive epithelial injury, cellular senescence, and aberrant fibroblast activation driven by TGF-beta, with each step offering distinct therapeutic opportunities [85]D5[94]D5[103]D5.
| Pathway | Key Mediators | Therapeutic Target |
|---|---|---|
| Epithelial injury and senescence | AEC apoptosis, SASP, CD38, mitochondrial dysfunction | Senolytics, antioxidants |
| Fibroblast activation | TGF-beta, WNT, PDGF, FGF, Src, S100A4 | Nintedanib, pirfenidone, integrin inhibitors |
| ECM remodeling | MMP-7, periostin, collagen | MMP inhibitors (investigational) |
| Vascular remodeling | EndoMT, BMPR2, endothelial dysfunction | Treprostinil (investigational) |
| Immune dysregulation | SPP1/MERTK macrophages, alarmins, IL-11 | Immunomodulators (investigational) |
3. Epidemiology, Etiology and Risk Factors
- ▸MUC5B rs35705950 confers OR 4-8 for IPF [85,136].
- ▸Cigarette smoking increases risk 2-3 fold; smoking cessation is key [53,215].
IPF incidence rises steeply with age, from <1 per 100,000 before 50 to >10 per 100,000 after 65 [169]A1a. A 2022 US analysis reported incidence of 1.5-2.0 per 100,000 and prevalence of 4.0-6.0 per 100,000 [57]B2b. Global age-standardized mortality ranges from 0.5 to 12 per 100,000 and has increased since 2001 [229]B2c[193]A1a. Men are affected ~1.5-2 times more often than women [228]B3b[213]D5. The strongest genetic risk factor is the MUC5B promoter variant rs35705950, conferring 4- to 8-fold increased risk in heterozygotes, present in ~30-40% of IPF patients vs. ~10% of controls [85]D5[136]D5[208]B3b. Rare variants in telomere-related genes (TERT, TERC, PARN, RTEL1) cause familial fibrosis [85]D5. Cigarette smoking is the dominant modifiable risk factor: ever-smokers have a 2- to 3-fold increased risk [53]D5[215]A1a. Occupational metal dust, wood dust, silica, and agricultural chemicals each increase risk by 1.5- to 2.5-fold; ~26% of cases are attributable to occupational inhalants [185]A1a. Chronic viral infections (EBV, CMV, HCV) have a 2- to 3-fold higher odds of IPF [175]A1a. GERD is present in 47% of patients [176]A1a[226]B2a. Air pollution (PM2.5, NO₂) increases both incident IPF and exacerbation risk [196]B2b[214]B2b. Diabetes mellitus shows a bidirectional Mendelian randomization association [210]B2b. Age-standardized mortality has risen 2- to 3-fold in high-income countries over the past two decades [229]B2c[193]A1a.
Pearl: IPF is a disease of aging with a strong genetic predisposition; the MUC5B promoter variant confers the highest known risk (OR 4-8), and smoking cessation remains the most impactful preventive measure [85]D5[215]A1a.
| Risk Factor | Odds Ratio / Relative Risk | Evidence Level |
|---|---|---|
| MUC5B rs35705950 (heterozygote) | OR 4.0-8.0 | Strong (multiple GWAS) [85]D5[136]D5 |
| Cigarette smoking (ever vs never) | OR 2.0-3.0 | Strong (meta-analysis) [53]D5[215]A1a |
| Occupational dust/metal exposure | OR 1.5-2.5 | Moderate (meta-analysis) [185]A1a |
| Viral infection (EBV, CMV, HCV) | OR 2.0-3.0 | Moderate (meta-analysis) [175]A1a |
| GERD | OR 1.5-2.0 | Moderate (meta-analysis) [176]A1a[226]B2a |
| Air pollution (PM2.5, NO₂) | HR 1.1-1.3 per 10 µg/m³ | Moderate (cohort) [196]B2b |
| Diabetes mellitus | OR 1.3-1.6 | Moderate (MR study) [210]B2b |
| Aging (per decade >50) | HR ~2.0 | Strong (epidemiology) [134]D5[169]A1a |
4. Clinical Presentation
- ▸Bilateral basilar crackles (>90% sensitive) and clubbing (25-50%) are key exam findings [33,194].
- ▸Acute exacerbation carries >50% 30-day mortality; early recognition is critical [21,266].
Dyspnoea on exertion is the dominant symptom, progressing over months to years [194]D5. Cough, often dry and paroxysmal, affects 50-84% and independently predicts progression and mortality [256]B2b[279]B2b. Diagnostic delay averages 1-2 years [286]B2b. Bilateral basilar inspiratory crackles (“Velcro-like”) are present in >90% at diagnosis [194]D5. Clubbing occurs in 25-50% and increases the likelihood of IPF over other fibrotic ILDs [33]D5. Signs of pulmonary (loud P2, right ventricular heave, peripheral oedema) emerge in advanced disease [194]D5. Phenotypic variants include rapid progressive (FVC decline >10%/year, ~15-20%), slow progressive (~30-40%), combined pulmonary fibrosis and emphysema (CPFE, ~10-15%), and familial (~5-10%) [60]B2b[287]B3b. Red flags: acute worsening over days to weeks with new GGO defines acute exacerbation (30-day mortality >50%) [21]D5[266]D5. Haemoptysis suggests lung cancer (complicates 5-10%) [292]B3b. Atypical presentations: incidental interstitial lung abnormalities on imaging (10-15%), predominant cough, acute exacerbation as first manifestation, or autoimmune features (IPAF) [250]B2b[255]D5[265]D5[278]D5. Chronic can mimic IPF; a thorough exposure history is essential [245]B2b.
Pearl: The combination of insidious exertional dyspnoea, dry cough, and bilateral basilar crackles in a patient over 60 years should prompt immediate consideration of IPF, as diagnostic delay is common and antifibrotic therapy is most effective when started early [194]D5[286]B2b.
| Finding | Sensitivity | Specificity | Clinical Pearl |
|---|---|---|---|
| Bilateral basilar crackles | >90% | Low (also in heart failure, bronchiectasis) | Persistent, unchanged by cough or position [194]D5 |
| Clubbing | 25-50% | High (rare in other chronic lung diseases) | More common in IPF than in non-IPF fibrotic ILD [33]D5 |
| Signs of pulmonary hypertension | Variable | Moderate | Loud P2, right ventricular heave, elevated JVP [194]D5 |
| Variant | Key Features | Frequency |
|---|---|---|
| Rapid progressive | FVC decline >10% predicted/year, high mortality | ~15-20% |
| Slow progressive | Indolent course, minimal functional decline over years | ~30-40% |
| Combined pulmonary fibrosis and emphysema (CPFE) | Preserved lung volumes, severe dyspnoea, high risk of pulmonary hypertension | ~10-15% |
| Familial pulmonary fibrosis | Younger age at onset, family history, telomere shortening | ~5-10% [60]B2b |
5. Diagnosis and Workup (Pulmonary Function and Imaging Anchored)
- ▸Definite UIP on HRCT in patient >60 with typical symptoms = diagnose IPF without biopsy [2,26].
- ▸FVC decline ≥10% predicted or DLCO decline ≥15% predicted within 12 months defines progression [254,301].
The diagnosis of IPF rests on a triad: compatible clinical context, restrictive ventilatory defect with impaired gas transfer, and a definitive UIP pattern on HRCT. Multidisciplinary discussion is the gold standard [2]A1c[3]A1c. Spirometry shows a restrictive pattern (FVC <80% predicted, FEV₁/FVC >0.70). DLCO is the most sensitive physiologic marker, often <60% predicted early; DLCO <40% identifies highest risk for early mortality [51]D5[206]B2b[254]B2b. The 6-minute walk test (6MWT) quantifies functional capacity; distance <250 m or desaturation <88% carries independent prognostic weight [204]D5[332]D5. Serial PFTs every 3-6 months track progression: an absolute FVC decline ≥10% predicted or DLCO decline ≥15% predicted within 12 months defines progressive disease and triggers therapeutic escalation [254]B2b[301]A1b. HRCT is the cornerstone of noninvasive diagnosis. A definite UIP pattern (subpleural, basal predominant reticulation with honeycombing) in a patient >60 with typical symptoms is sufficient to diagnose IPF without biopsy [2]A1c[26]D5. If HRCT shows probable UIP, indeterminate, or alternative patterns, lung biopsy is indicated. Transbronchial lung cryobiopsy (TBLC) is first-line; the COLDICE trial showed diagnostic agreement of 70.7% with surgical lung biopsy [164]B2b. TBLC has a pooled diagnostic yield of 80%, pneumothorax rate of 9%, and moderate bleeding in 5% [307]A1a. Laboratory studies help exclude alternative causes: serology for connective tissue disease, hypersensitivity pneumonitis panel, and BAL (lymphocytosis >30% suggests HP) [308]A1a[311]B2b[317]B3b. MUC5B genotyping is not required for diagnosis but may aid in familial cases [331]D5.
Pearl: A definite UIP pattern on HRCT in a patient aged >60 years with typical symptoms is sufficient to diagnose IPF without biopsy; multidisciplinary discussion remains the gold standard to avoid misclassification in atypical cases [2]A1c[26]D5.
| Pattern | Key Features | Diagnostic Implication |
|---|---|---|
| UIP | Subpleural, basal predominant reticular abnormality with honeycombing (traction bronchiectasis may be present) | Definite IPF; no biopsy needed |
| Probable UIP | Subpleural, basal predominant reticular abnormality with traction bronchiectasis but no honeycombing | Biopsy recommended if clinical context supports |
| Indeterminate | Subpleural, basal predominant ground-glass or fine reticulation without honeycombing or traction bronchiectasis | Biopsy required for diagnosis |
| Alternative | Upper lobe predominance, cysts, nodules, mosaic attenuation, or pleural plaques | Suggests alternative ILD (e.g., hypersensitivity pneumonitis, sarcoidosis) |
| Test | Finding | Purpose |
|---|---|---|
| Serology for connective tissue disease | ANA, RF, anti-CCP, anti-Scl-70, anti-Ro52 | Exclude CTD-ILD (e.g., RA-ILD, SSc-ILD) [311]B2b[317]B3b |
| Hypersensitivity pneumonitis panel | Precipitins to avian, fungal, or other antigens | Exclude chronic HP [308]A1a |
| Bronchoalveolar lavage (BAL) | Lymphocytosis >30% suggests HP; neutrophilia/eosinophilia may be seen in IPF but is nonspecific | Used selectively when HP or infection is suspected [308]A1a |
| MUC5B rs35705950 genotyping | T allele increases IPF risk 3-6 fold | Not required for diagnosis; may aid in familial cases [331]D5 |
6. Severity, Staging and Risk Stratification
- ▸GAP index stages I/II/III have 1-year mortality of 6%/16%/39% [190].
- ▸FVC decline ≥10% predicted in 12 months increases mortality risk ~2.5-fold [206].
Median survival in IPF is 3-5 years from diagnosis, but individual trajectories vary [190]D5[193]A1a. The GAP (Gender-Age-Physiology) index is the most widely validated prognostic tool, stratifying patients into stages I (0-3 points), II (4-5), and III (6-8) with 1-year mortality of 6%, 16%, and 39%, respectively [3]A1c[190]D5. The ILD-GAP model extends this to other fibrotic ILDs with a C-statistic of 0.69-0.74 for 1-year mortality [205]B2b. Serial lung function thresholds are key: FVC decline ≥10% predicted within 12 months increases mortality risk (HR 2.1-3.0) [206]B2b[349]B2b; DLCO decline ≥15% predicted carries similar weight (HR 2.5) [206]B2b. The combination of both declines identifies a very high-risk subgroup with 2-year mortality >50% [206]B2b. Circulating biomarkers add independent information: MMP-7 >4.2 ng/mL predicts 1-year disease progression (OR 2.8) [224]B2b. Prostasin is independently associated with mortality (HR 1.4 per log-unit increase) in the IPF-PRO Registry [230]B3b. In the ISABELA trial, a 17-biomarker panel improved discrimination for 1-year progression (AUC 0.72 vs. 0.65 for GAP alone) [188]B2b. Imaging-based staging: reticulation plus honeycombing extent >20% of lung volume predicts mortality (HR 1.8) [6]A1c. The presence of a UIP pattern on HRCT, even without histologic confirmation, confers worse prognosis [212]B2b. CPFE phenotype has higher mortality (HR 1.6) due to pulmonary and lung cancer [314]B2b. Sarcopenia and dynapenia are prevalent (30-50%) and independently predict mortality (HR 2.1 for dynapenia) [222]B2b. Nutritional assessment identifies malnourished patients (25% of IPF cohort) with 2-year mortality of 40% vs. 15% in well-nourished [283]B2b. Neighborhood disadvantage is associated with 30% higher mortality [281]B3b.
Pearl: The GAP index and serial FVC decline ≥10% predicted are the most validated tools for IPF risk stratification; integrating biomarkers (MMP7, prostasin) and imaging (UIP pattern extent) improves discrimination and guides transplant referral timing [190]D5[206]B2b[224]B2b.
| Variable | Points |
|---|---|
| Sex | Female = 0, Male = 1 |
| Age (years) | ≤60 = 0, 61-65 = 1, >65 = 2 |
| FVC % predicted | >75 = 0, 50-75 = 1, <50 = 2 |
| DLCO % predicted | >55 = 0, 36-55 = 1, ≤35 = 2, unable = 3 |
| Stage | Total Score |
| I | 0-3 |
| II | 4-5 |
| III | 6-8 |
Data from [3]A1c[190]D5
| Parameter | Threshold | Mortality Risk | Reference |
|---|---|---|---|
| FVC decline | ≥10% predicted in 12 months | HR 2.1-3.0 | [206]B2b[349]B2b |
| DLCO decline | ≥15% predicted in 12 months | HR 2.5 | [206]B2b |
| MMP7 | >4.2 ng/mL | OR 2.8 for progression | [224]B2b |
| Prostasin | Per log-unit increase | HR 1.4 | [230]B3b |
| UIP pattern on HRCT | Present vs. absent | 5-year TFS 45% vs. 65% | [212]B2b |
| CPFE phenotype | Emphysema + fibrosis | HR 1.6 vs. IPF alone | [314]B2b |
| Dynapenia | Low handgrip strength | HR 2.1 | [222]B2b |
7. Acute Management and Exacerbation Rescue
- ▸AE-IPF: high-dose methylprednisolone (500-1000 mg IV × 3 days) is first-line [15,21].
- ▸Do NOT add cyclophosphamide (EXAFIP trial: no benefit, trend to harm) [162].
An acute respiratory deterioration in a patient with IPF demands immediate triage to distinguish acute exacerbation (AE-IPF) from infection, PE, aspiration, pneumothorax, heart failure, and drug toxicity [15]D5[277]D5. AE-IPF is defined by acute worsening, new bilateral GGO/consolidation on HRCT, and no identifiable trigger [15]D5. Annual incidence is 5-15%; in-hospital mortality exceeds 50% [21]D5[63]D5[174]A1a[380]A1a. Step 1: Initial assessment - measure SpO₂, ABG (PaO₂/FiO₂), HRCT, CBC, comprehensive metabolic panel, troponin, NT-proBNP. Bronchoscopy with BAL is strongly recommended to identify infection [387]B3b. Classify severity by PaO₂/FiO₂: Mild >200, Moderate 150-200, Severe <150. Step 2: First-line intervention - high-dose IV methylprednisolone 500-1000 mg IV daily for 3 days, then oral prednisolone taper over 4-6 weeks [15]D5[21]D5[277]D5. This is expert consensus; no placebo-controlled RCT confirms superiority [380]A1a. Step 3: Do NOT add cyclophosphamide - the EXAFIP trial found no survival benefit and trend toward harm (HR 1.32) [162]A1b. Do NOT use anticoagulation [354]B3b. Provide broad-spectrum antibiotics empirically; stop if BAL cultures negative at 48-72 h [21]D5. Consider IVIG (ongoing MERCURION-IPF trial) [37]A1b. Consider recombinant thrombomodulin (experimental, not approved) [371]B3b. Step 4: Oxygen and ventilation - target SpO₂ ≥88%. Start with nasal cannula; escalate to high-flow nasal cannula if PaO₂/FiO₂ <200 [393]A1a. Use NIV for hypercapnia (PaCO₂ >45) or acidosis (pH <7.35) [216]D5. Invasive mechanical ventilation (lung-protective strategy) for refractory hypoxemia; in-hospital mortality with IMV approaches 70-90% [216]D5[380]A1a. Step 5: Resolution - after 72 hours, if improved, transition to oral prednisolone taper. Continue background antifibrotic therapy [174]A1a. Re-evaluate lung transplant candidacy in all survivors. Treatment failure - re-image, repeat BAL, offer palliative care, consider ECMO only if transplant candidate.
Pearl: In acute exacerbation of IPF, start high-dose methylprednisolone (500-1000 mg IV daily × 3 days) with broad-spectrum antibiotics and supportive oxygen; do NOT add cyclophosphamide (EXAFIP trial [162]A1b) and do NOT use anticoagulation; early consideration of lung transplant referral and goals-of-care discussions is essential given in-hospital mortality exceeding 50% [15]D5[21]D5[174]A1a[380]A1a.
| Drug | Starting Dose | Target / Max Dose | Renal Adjustment | Hepatic Adjustment | Key Monitoring |
|---|---|---|---|---|---|
| Methylprednisolone IV | 500-1000 mg daily × 3 days | Then oral prednisolone taper | None | None | Blood glucose, BP, electrolytes [15]D5[380]A1a |
| Cyclophosphamide IV | 600 mg/m² days 0, 15, 30, 60 | Same | eGFR < 20: avoid | Child-Pugh B/C: avoid | CBC, urinalysis (hemorrhagic cystitis) [162]A1b |
| Nintedanib (oral) | 150 mg PO twice daily | 150 mg twice daily | No adjustment | Child-Pugh B: 100 mg twice daily; C: avoid | LFTs, GI side effects [174]A1a |
| Pirfenidone (oral) | 267 mg (1 capsule) three times daily | 801 mg (3 capsules) three times daily | No adjustment | Consider dose reduction | LFTs, photosensitivity [240]A1b |
8. Long-term and Definitive Management
- ▸Nintedanib 150 mg BID or pirfenidone 2403 mg/day reduces FVC decline by ~50% [240,343].
- ▸Refer for lung transplant when disease progresses despite antifibrotics, DLCO <39%, or 6MWT <250 m [202].
Antifibrotic therapy with nintedanib or pirfenidone is the cornerstone of long-term disease modification, reducing FVC decline by approximately 50% over 52 weeks [240]A1b[343]A1b. Nintedanib 150 mg twice daily targets PDGFR, FGFR, and VEGFR. In the INPULSIS trials, it reduced annual FVC decline from -223.5 mL/year to -113.6 mL/year (difference 109.9 mL/year) [343]A1b. A meta-analysis showed a reduction in all-cause mortality (HR 0.70) [174]A1a. Pirfenidone 2403 mg/day (three 267-mg capsules TID) has anti-inflammatory and antioxidant properties. In ASCEND, it reduced the proportion with ≥10% FVC decline or death from 31.8% to 16.5% (NNT=7) [240]A1b. The CleanUP-IPF study found no significant difference in 12-month FVC decline between the two agents [167]B2b. Selection is guided by tolerability and comorbidities. Emerging therapies: Nerandomilast (PDE4B inhibitor, 18 mg BID) showed FVC benefit of 95.7 mL at 52 weeks in FIBRONEER-IPF [364]A1b. Inhaled treprostinil (12 breaths QID) showed FVC benefit of 50.1 mL in TETON-2 and reduced acute exacerbations [113]A1b[114]A1b. Bexotegrast (αvβ6/αvβ1 integrin inhibitor, 320 mg QD) showed FVC benefit of 60 mL at 12 weeks [159]A1b. Admilparant (LPA1 antagonist, 60 mg BID) reduced FVC decline by 90 mL/year [158]A1b. Non-pharmacologic management: Pulmonary rehabilitation improves 6-minute walk distance by 30.5 m (Cochrane review) [273]A1a. Long-term oxygen therapy (LTOT) for resting hypoxemia (SpO₂ ≤88% or PaO₂ ≤55 mmHg) reduces acute exacerbations by 40% [377]B2b. Lung transplantation is the only definitive therapy improving survival; refer early when disease progresses despite antifibrotic therapy, DLCO <39% predicted, or 6-minute walk distance <250 m [202]D5. What NOT to do: Do NOT use combination therapy with prednisone, azathioprine, and N-acetylcysteine (PANTHER trial: increased mortality) [366]A1b. Do NOT use N-acetylcysteine monotherapy [303]A1b. Do NOT use immunosuppressants outside acute exacerbation trials [443]D5.
Pearl: Initiate nintedanib or pirfenidone at diagnosis to slow FVC decline (NNT=7 for pirfenidone to prevent ≥10% decline or death); refer for lung transplantation early when disease progresses despite antifibrotic therapy, and avoid immunosuppressants which increase mortality in IPF [240]A1b[366]A1b[202]D5.
| Drug | Starting dose | Target / max dose | Renal adjustment | Hepatic adjustment | Key monitoring |
|---|---|---|---|---|---|
| Nintedanib | 150 mg PO BID | 150 mg BID | No adjustment for eGFR ≥30; avoid if <30 | Child-Pugh A: 100 mg BID; Child-Pugh B/C: avoid | LFTs (ALT/AST) monthly for 3 months, then q3mo; diarrhea management |
| Pirfenidone | 267 mg PO TID (week 1), escalate to 534 mg TID (week 3) | 801 mg TID (2403 mg/day) | No adjustment | Child-Pugh A: 267 mg BID; Child-Pugh B: avoid | LFTs monthly for 6 months, then q3mo; photosensitivity precautions |
| Option | Indication / Line | Dose or Specifics | Key Trial | Outcome | Evidence Level |
|---|---|---|---|---|---|
| Nintedanib | First-line | 150 mg BID | INPULSIS [343]A1b | FVC decline reduced by 109.9 mL/year | 1b |
| Pirfenidone | First-line | 2403 mg/day | ASCEND [240]A1b | NNT=7 to prevent ≥10% FVC decline or death | 1b |
| Nerandomilast | Second-line / emerging | 18 mg BID | FIBRONEER-IPF [364]A1b | FVC benefit 95.7 mL at 52 weeks; AE/hospitalization/death HR 0.67 | 1b |
| Inhaled treprostinil | Adjunctive (PH-ILD) | 12 breaths QID | TETON-2 [113]A1b | FVC benefit 50.1 mL at 52 weeks; AE HR 0.64 | 1b |
| Bexotegrast | Emerging | 320 mg QD | INTEGRIS-IPF [159]A1b | FVC benefit 60 mL at 12 weeks | 1b |
| Admilparant | Emerging | 60 mg BID | Phase 2 [158]A1b | FVC decline reduced by 90 mL/year | 1b |
9. Inhaled and Oxygen Therapy and the Exacerbation Pathway (Specialty-Distinctive)
- ▸LTOT for resting SpO₂ ≤88% reduces hospitalizations (rate ratio 0.79) [377].
- ▸AE-IPF: pulse methylprednisolone 500-1000 mg IV × 3 days is standard; do NOT add cyclophosphamide [15,162].
Once antifibrotic therapy is established, managing gas exchange and detecting acute exacerbations is critical. Step 1: Initial assessment - classify by resting and exertional SpO₂. Resting SpO₂ <88% (or PaO₂ <55 mmHg) defines chronic resting hypoxemia and mandates long-term oxygen therapy (LTOT) [452]B2b. Exertional desaturation <88% on 6MWT identifies candidates for ambulatory oxygen [450]A1b. Nocturnal hypoxemia (≥10% of sleep time with SpO₂ <90%) should be screened with overnight oximetry [12]B2a. Step 2: LTOT - prescribed for ≥15 hours/day to maintain SpO₂ ≥90%, typically 1-3 L/min via nasal cannula. The DISCOVERY cohort showed LTOT reduced respiratory-related hospitalizations (rate ratio 0.79) [377]B2b. Do NOT use supplemental oxygen in normoxic patients without exertional desaturation [381]A1a. Step 3: Acute exacerbation rescue algorithm - AE-IPF defined by acute dyspnea, new bilateral GGO on HRCT, and no alternative cause [15]D5. Pulse methylprednisolone 500-1000 mg IV daily for 3 days is standard [15]D5. The EXAFIP trial showed no benefit from adding cyclophosphamide [162]A1b. Recombinant thrombomodulin alfa failed to improve survival in a phase 3 trial [445]A1b. Do NOT use inhaled carbon monoxide or BG00011 [446]A1b[449]A1b. Step 4: Monitoring and transition - daily PaO₂/FiO₂ ratio, pH, PaCO₂. Failure to improve PaO₂/FiO₂ by >50 points by day 3 is a poor sign. The H.A.L. score (Honeycombing, Age >75, LDH >222 U/L) predicts AE-IPF risk [460]B2b. If intubated, use lung-protective ventilation (tidal volume 6 mL/kg, plateau ≤30 cmH₂O). The 180-day mortality in mechanically ventilated AE-IPF exceeds 70% [397]B2b. When stabilized, transition to conventional oxygen and then home LTOT. Early lung transplant referral is indicated in survivors [395]B3b.
Pearl: Long-term oxygen therapy reduces hospitalisations in IPF with resting hypoxemia (NNT = 8 to prevent one annual hospitalisation) [377]B2b (2b); for acute exacerbation, pulse methylprednisolone is standard but carries no proven survival benefit, making early transplant referral and advance care planning critical for any hospitalised patient who survives the first 48 hours [162]A1b [380]A1a.
| Oxygen Modality | Indication | Device | Flow / Settings | Evidence Level |
|---|---|---|---|---|
| Ambulatory oxygen | Isolated exertional desaturation < 88% on 6MWT | Portable oxygen concentrator (POC) or cylinder | 2-6 L/min during exertion; trial may improve dyspnoea but not survival | 1b [450]A1b |
| Nocturnal oxygen | Nocturnal hypoxemia (≥10% TST < 90%) | Nasal cannula | 1-3 L/min overnight; target SpO₂ ≥ 90% | 2a [12]B2a |
| Long-term oxygen therapy (LTOT) | Resting PaO₂ ≤ 55 mmHg or SpO₂ ≤ 88% | Concentrator + stationary cannula | ≥ 15 h/day; titrate to SpO₂ ≥ 90% | 2b [377]B2b |
| Drug / Intervention | Typical dose | Indication | Key evidence | Outcome |
|---|---|---|---|---|
| Methylprednisolone (pulse) | 500-1000 mg IV x 3 days | First-line for all AE-IPF | 90-day mortality 42% (95% CI 19-67) [380]A1a (1a) | No survival benefit over lower dose; used as standard |
| Cyclophosphamide | 600 mg/m² IV days 0, 15, 30, 60 | Not recommended | EXAFIP: HR 1.32 (0.76-2.30) [162]A1b (1b) | No benefit; trend to harm |
| Thrombomodulin alfa | 380 U/kg/day SC x 6 days | Not recommended | Phase 3: HR 1.68 (0.75-3.76) [445]A1b (1b) | No survival benefit |
| IVIG | 0.4 g/kg/day x 5 days | Investigational | MERCURION-IPF (ongoing phase 3) [37]A1b (1b) | Primary endpoint: in-hospital mortality (data pending) |
10. Complications
- ▸Pulmonary hypertension develops in 30-50% of advanced IPF and worsens prognosis [342,485].
- ▸Lung cancer risk is elevated (3-48%): squamous cell and adenocarcinoma are common [342,495].
Respiratory failure accounts for 40-60% of deaths in IPF [190]D5. Pulmonary (PH) is common in advanced disease (30-50%) and is associated with worse survival [342]B2a[315]D5[485]D5. Screening with echocardiography is recommended in all lung transplant candidates [492]D5. Inhaled treprostinil improved exercise capacity and FVC in the INCREASE trial [237]B2b. Sildenafil added to pirfenidone did not improve outcomes [165]A1b. Bosentan and ambrisentan are not recommended due to lack of efficacy and potential harm [358]C4[482]A1b. Pneumothorax occurs in ~5% of IPF patients, often requiring chest tube drainage or pleurodesis if recurrent [509]C4. Pleuroparenchymal fibroelastosis (PPFE) can coexist and increases pneumothorax risk [496]C4. Lung cancer develops in 3-48% of IPF patients, predominantly squamous cell carcinoma and adenocarcinoma [342]B2a[495]C4. Management is complicated by high rates of treatment-related pneumonitis and poor pulmonary reserve [508]B3b. Venous thromboembolism (VTE) prevalence is 5-10%; hospitalized IPF patients should receive prophylactic LMWH (e.g., enoxaparin 40 mg SC daily) unless contraindicated [489]D5. Other complications: atrial fibrillation (up to 20%), gastroesophageal reflux (30-90%), obstructive sleep apnea (6-91%), and depression (20-30%) [125]D5[252]A1a[342]B2a. Pain management: for refractory dyspnea, low-dose opioids (e.g., morphine 2.5-5 mg orally q4h PRN) are effective [276]A1a. Benzodiazepines have not shown benefit [48]A1a. Pulmonary rehabilitation after stabilization of acute exacerbations improves 6-minute walk distance and quality of life [241]A1b. Early mobilization, oral care, and minimizing indwelling catheters are key to preventing hospital-acquired complications.
Pearl: Respiratory failure remains the most common cause of death in IPF, but proactive management of pulmonary hypertension, lung cancer, and venous thromboembolism can improve quality of life and survival [190]D5[342]B2a[489]D5.
| Complication | Frequency | Prevention | Management |
|---|---|---|---|
| Respiratory failure | 40-60% of deaths [190]D5 | Antifibrotic therapy, LTOT | NIV, invasive ventilation if appropriate |
| Pulmonary hypertension | 30-50% advanced [342]B2a[485]D5 | Screen with echo | Inhaled treprostinil, LTOT, consider PDE5i |
| Pneumothorax | ~5% [509]C4 | Avoid high airway pressures | Chest tube, pleurodesis if recurrent |
| Lung cancer | 3-48% [342]B2a | Smoking cessation | Multidisciplinary, cautious surgery/RT |
| Venous thromboembolism | 5-10% [489]D5 | LMWH prophylaxis | Anticoagulation |
| Gastroesophageal reflux | 30-90% [252]A1a | PPI, consider fundoplication | Antacids, surgery in selected |
| Obstructive sleep apnea | 6-91% [342]B2a | Screen with polysomnography | CPAP |
| Depression | 20-30% [125]D5 | Screen with PHQ-9 | Counseling, SSRIs |
11. Prognosis and Natural History
- ▸Untreated IPF median survival 2-3 years; antifibrotics reduce FVC decline by ~50% [240,343].
- ▸Antifibrotic therapy reduces all-cause mortality by ~30% (HR 0.70; NNT=22 over 12 months) [174].
Untreated IPF has a median survival of 2 to 3 years from diagnosis, with respiratory failure as the most common cause of death [190]D5. The annual FVC decline in untreated patients is approximately 150-200 mL/year [240]A1b[343]A1b. Acute exacerbations occur at an annual incidence of 5-10% and carry an in-hospital mortality of 50% or more [15]D5. Antifibrotic therapy with pirfenidone or nintedanib slows FVC decline by approximately 50%. A pooled analysis of pirfenidone trials showed a 48% relative reduction in all-cause mortality over 120 weeks (HR 0.52; NNT = 28) [171]A1a. A meta-analysis of antifibrotic therapy confirmed a 30% reduction in all-cause mortality (HR 0.70; NNT = 22 over 12 months) and a 64% reduction in acute exacerbation risk (HR 0.36; NNT = 25) [174]A1a. Long-term extension studies (INPULSIS-ON) show sustained treatment effect for up to 4 years [360]C4. Factors affecting prognosis: baseline FVC <50% predicted or DLCO <35% predicted gives median survival <2 years [190]D5[206]B2b. A ≥10% decline in FVC at 12 months increases mortality risk 2.5-fold [206]B2b. Pulmonary hypertension develops in up to 40% of advanced IPF, with median survival <1 year when present [342]B2a. CPFE has mortality similar to or worse than IPF alone [314]B2b. Genetic factors: MUC5B carriers have slower progression; telomere-related gene variants are associated with more rapid decline [85]D5[305]B2b. Leukocyte telomere length <10th percentile for age independently predicts mortality (HR 2.2) [513]B3b. The GAP index stratifies 1-year mortality at 6% (stage I), 16% (stage II), and 39% (stage III) [205]B2b. The TAP index adds telomere length and improves discrimination [513]B3b.
Pearl: Untreated IPF has a median survival of 2-3 years, but antifibrotic therapy reduces FVC decline by ~50% and all-cause mortality by ~30% (NNT = 22 over 12 months); the GAP index and telomere length are the most clinically useful tools for individual risk stratification [171]A1a[174]A1a[190]D5[205]B2b.
| Model | Components | 1-Year Mortality by Stage |
|---|---|---|
| GAP | Gender, Age, FVC, DLCO | Stage I: 6%; Stage II: 16%; Stage III: 39% [205]B2b |
| ILD-GAP | Same + ILD subtype | Validated across non-IPF ILDs [205]B2b |
| TAP | Telomere length, Age, FVC, DLCO | Superior to GAP in telomere-shortened patients [513]B3b |
12. Special Populations and Prevention
- ▸In children with UIP pattern, test for MARS1, SFTPC, and telomere gene mutations [298,553].
- ▸Smoking cessation and avoiding occupational dusts are key primary prevention strategies [185].
Pediatrics: IPF is exceptionally rare in children; when a UIP pattern appears, genetic testing for MARS1, SFTPC, and telomere-related genes is essential [298]D5[553]D5. Nintedanib in children aged 6-17 years with progressive-fibrosing ILD uses weight-based dosing: 50 mg BID for 6-11 years and 100 mg BID for 12-17 years [538]B2b. Prognosis varies by underlying etiology; some require lung transplantation [298]D5. Pregnancy: IPF is uncommon in women of childbearing age, but carries substantial risks. Nintedanib and pirfenidone are contraindicated during pregnancy due to teratogenicity. Antifibrotic therapy should be stopped before conception. Multidisciplinary management including maternal-fetal medicine is required. Vaginal delivery with epidural anesthesia is preferred if oxygenation is adequate. Elderly: Most patients are diagnosed after age 70. Diagnostic challenges include comorbidities (e.g., heart failure, chronic HP) [530]B2b. Surgical lung biopsy carries higher risk in patients >75 years (30-day mortality 1.7%) [524]B3b[549]B3b. Antifibrotic therapy is safe and effective in patients >80 years, though dose adjustment may be needed for declining renal function [541]B3b[415]D5. Frailty assessment should guide treatment intensity [415]D5. Immunocompromised host: IPF itself is not immunocompromised, but impaired mucociliary clearance increases infection risk. Vaccination against influenza, pneumococcus, and SARS-CoV-2 is strongly recommended [458]D5. Prevention: Primary prevention focuses on smoking cessation and avoiding occupational dusts [185]A1a. Long-term PM2.5 and NO₂ exposure increases mortality [525]B2b. Secondary prevention: first-degree relatives of familial pulmonary fibrosis patients have a 31% prevalence of interstitial lung abnormalities on CT screening [520]C4. Routine screening is not recommended [535]B2b. Lung cancer screening CT incidentally identifies interstitial lung abnormalities in 1.5% of participants, offering an opportunity for early diagnosis [536]B3b. Exacerbation prevention includes consistent antifibrotic therapy (reduces risk by ~50%), vaccination, and prompt treatment of infections [63]D5. The role of proton pump inhibitors for GERD remains uncertain [519]B2b.
Pearl: In elderly patients with IPF, antifibrotic therapy is safe and effective regardless of age, but comorbidity management and frailty assessment are essential; in children, genetic testing for MARS1 and surfactant mutations should precede treatment decisions, and nintedanib dosing is weight-based [538]B2b[541]B3b[553]D5.
| Population | Diagnostic Modifications | Treatment Modifications | Prognosis |
|---|---|---|---|
| Pediatrics | Genetic testing (MARS1, SFTPC); MDD essential | Nintedanib weight-based (50 mg BID age 6-11; 100 mg BID age 12-17) [538]B2b | Variable; may require transplant |
| Pregnancy | Avoid HRCT if possible; use lung US or low-dose CT | Stop antifibrotics before conception; supportive care only | High risk of maternal decompensation |
| Elderly | HRCT often sufficient; avoid SLB if possible | Standard doses; adjust for renal function; assess frailty [415]D5 | Comorbidity-driven; median survival 3-5 years |
| Immunocompromised | Screen for opportunistic infections | Vaccinate; avoid live vaccines; prompt antibiotic/antiviral therapy | Infection is a common trigger for AE-IPF |
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