GITNUXREPORT 2026

Pulmonary Fibrosis Statistics

Despite being rare, this aging-related lung disease carries an eighty percent five-year mortality rate.

Sarah Mitchell

Sarah Mitchell

Senior Researcher specializing in consumer behavior and market trends.

First published: Feb 13, 2026

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Key Statistics

Statistic 1

Dry cough present in 70-85% of IPF patients.

Statistic 2

Progressive dyspnea in 90% at diagnosis.

Statistic 3

Bibasilar crackles in 80-90% on exam.

Statistic 4

Median FVC decline 180-250 ml/year.

Statistic 5

6MWD average 250-350 meters at diagnosis.

Statistic 6

Clubbing in 25-50% of cases.

Statistic 7

HRCT usual interstitial pneumonia (UIP) pattern in 50-60%.

Statistic 8

DLCO <40% predicted in 50% advanced IPF.

Statistic 9

Weight loss in 20-30% patients.

Statistic 10

Cyanosis in 50% at rest in severe disease.

Statistic 11

Fatigue reported by 70%.

Statistic 12

SGRQ score average 45-50 in IPF.

Statistic 13

Acute exacerbations in 5-10% per year.

Statistic 14

Chest X-ray abnormal in 90%.

Statistic 15

BAL lymphocytosis <15% in UIP.

Statistic 16

GAP index stage I: 6% 1-yr mortality.

Statistic 17

Velcro rales on auscultation hallmark.

Statistic 18

Desaturation <88% on 6MWT in 50%.

Statistic 19

Composite physiologic index >50 in 40%.

Statistic 20

Orthodeoxia in 20% advanced cases.

Statistic 21

MRC dyspnea score 3-4 in 60%.

Statistic 22

HRCT honeycombing in 90% definite UIP.

Statistic 23

Finger clubbing OR 5.37 for IPF diagnosis.

Statistic 24

PFT restriction: FVC <80% in 90%.

Statistic 25

Echo right ventricular systolic pressure >50 mmHg in 30%.

Statistic 26

LDH elevated in 70% acute exacerbations.

Statistic 27

KL-6 biomarker elevated in 80%.

Statistic 28

Idiopathic pulmonary fibrosis (IPF) prevalence in the US is 14-43 per 100,000.

Statistic 29

Global IPF incidence is 0.6-4.1 per 100,000 person-years.

Statistic 30

IPF incidence in Europe ranges from 1.3-10.7 per 100,000.

Statistic 31

UK IPF prevalence increased from 20.1 to 29.8 per 100,000 between 1991-2003.

Statistic 32

Male predominance in IPF: 1.6-2.4:1 male-to-female ratio.

Statistic 33

IPF median age at diagnosis is 66 years.

Statistic 34

IPF accounts for 50% of pulmonary fibrosis cases.

Statistic 35

Familial IPF represents 2-20% of cases.

Statistic 36

IPF prevalence in Japan: 5.39 per 100,000.

Statistic 37

US IPF hospitalizations rose 135% from 2001-2011.

Statistic 38

IPF mortality in US: 6.8-8.6 per 100,000.

Statistic 39

IPF prevalence in Olmsted County, MN: 42.7 per 100,000.

Statistic 40

Global PF prevalence: 13-20 per 100,000.

Statistic 41

IPF cases in US estimated at 50,000-80,000.

Statistic 42

Incidence in Australia: 2.3 per 100,000.

Statistic 43

IPF more common in Caucasians.

Statistic 44

Annual IPF diagnoses in Europe: ~13,000.

Statistic 45

Prevalence doubles every decade after 55 years.

Statistic 46

IPF in US males: 20.25 per 100,000.

Statistic 47

IPF in US females: 13.51 per 100,000.

Statistic 48

Pediatric PF incidence: <1 per 100,000.

Statistic 49

IPF mortality doubled from 1996-2007 in US.

Statistic 50

Prevalence in Canada: 18.4 per 100,000.

Statistic 51

IPF incidence in Spain: 2.4 per 100,000.

Statistic 52

Higher prevalence in rural areas.

Statistic 53

IPF cases projected to rise 11% by 2025 in US.

Statistic 54

Familial clustering in 2.2-3.7% of cases.

Statistic 55

IPF prevalence in Israel: 4.5 per 100,000.

Statistic 56

Incidence in New Zealand: 4.1 per 100,000.

Statistic 57

80% of IPF patients die within 5 years of diagnosis.

Statistic 58

Median survival 3-5 years from diagnosis.

Statistic 59

1-year mortality 10-15%.

Statistic 60

GAP stage III: 39% 1-year mortality.

Statistic 61

Lung transplant 5-year survival 50-60%.

Statistic 62

Acute exacerbation mortality 50% per event.

Statistic 63

FVC decline >10% predicts 1-year mortality 40%.

Statistic 64

DLCO <35% predicted: median survival 1.5 years.

Statistic 65

DES score >4: poor prognosis.

Statistic 66

Male gender HR 1.3 for mortality.

Statistic 67

UIP pattern on HRCT better prognosis than NSIP.

Statistic 68

Age >65 HR 1.8.

Statistic 69

Pulmonary hypertension present in 30-50%, worsens survival.

Statistic 70

6MWD <250m: 1-year mortality 24%.

Statistic 71

Hospitalization mortality 20-30%.

Statistic 72

Nintedanib slows FVC decline by 107 ml/year.

Statistic 73

Pirfenidone reduces mortality risk 48% at 1 year.

Statistic 74

Oxygen therapy improves survival by 50%.

Statistic 75

BMI <21 kg/m2 HR 1.58.

Statistic 76

Composite GAP index c-statistic 0.80 for mortality.

Statistic 77

Post-transplant survival median 4.5 years.

Statistic 78

Annual FVC decline predicts 2-year mortality AUROC 0.73.

Statistic 79

Serum SP-D >100 ng/ml poor prognosis.

Statistic 80

Right heart failure in 20%, median survival 8 months.

Statistic 81

Lung cancer co-occurrence 10-20%, worsens survival.

Statistic 82

CPI >70: median survival 11 months.

Statistic 83

5-year survival post-diagnosis 20-40%.

Statistic 84

Antifibrotic therapy improves 1-year survival to 95%.

Statistic 85

GAP stage II: 16% 1-year mortality.

Statistic 86

Pirfenidone slows FVC decline 110 ml/year.

Statistic 87

Nintedanib reduces acute exacerbations by 38%.

Statistic 88

Cigarette smoking associated with 48% of IPF cases.

Statistic 89

Metal dust exposure increases IPF risk by 2.5-fold.

Statistic 90

Familial history raises risk 3.3-13-fold.

Statistic 91

Gastroesophageal reflux disease (GERD) in 87% of IPF patients.

Statistic 92

Smoking history in 63% of IPF cases.

Statistic 93

Asbestos exposure linked to 5-10% of cases.

Statistic 94

Genetic mutations (MUC5B) increase risk 3-7 fold.

Statistic 95

Wood dust exposure OR 1.95 for IPF.

Statistic 96

Hypersensitivity pneumonitis as precursor in 10-15%.

Statistic 97

Telomerase mutations in 1-8% familial IPF.

Statistic 98

Chronic aspiration risk factor in 30-90%.

Statistic 99

Farming occupation OR 1.3-2.0.

Statistic 100

TERT gene mutation risk RR 2.7.

Statistic 101

Obesity BMI>30 reduces IPF risk OR 0.7.

Statistic 102

Viral infections (EBV, CMV) associated in 20-40%.

Statistic 103

Stone cutting/polishing OR 2.49.

Statistic 104

Emphysema co-occurrence in 30-42%.

Statistic 105

Autoimmune disease overlap in 10-30%.

Statistic 106

Hairdressing occupation risk increased.

Statistic 107

SFTPC mutations in familial PF.

Statistic 108

Socioeconomic status inverse association.

Statistic 109

Welding fumes OR 1.8.

Statistic 110

Connective tissue disease in 15% IPF.

Statistic 111

MUC5B promoter variant in 35% IPF.

Statistic 112

Pesticide exposure OR 1.6.

Statistic 113

Raynaud's phenomenon in 15-20%.

Statistic 114

ABCA3 mutations rare cause.

Sources
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Beneath every breath is a silent epidemic, and the staggering reality of pulmonary fibrosis, which claims the lives of 80% of patients within five years of diagnosis, demands our urgent attention.

Key Takeaways

  • Idiopathic pulmonary fibrosis (IPF) prevalence in the US is 14-43 per 100,000.
  • Global IPF incidence is 0.6-4.1 per 100,000 person-years.
  • IPF incidence in Europe ranges from 1.3-10.7 per 100,000.
  • Cigarette smoking associated with 48% of IPF cases.
  • Metal dust exposure increases IPF risk by 2.5-fold.
  • Familial history raises risk 3.3-13-fold.
  • Dry cough present in 70-85% of IPF patients.
  • Progressive dyspnea in 90% at diagnosis.
  • Bibasilar crackles in 80-90% on exam.
  • 80% of IPF patients die within 5 years of diagnosis.
  • Median survival 3-5 years from diagnosis.
  • 1-year mortality 10-15%.

Despite being rare, this aging-related lung disease carries an eighty percent five-year mortality rate.

Clinical Presentation and Diagnosis

  • Dry cough present in 70-85% of IPF patients.
  • Progressive dyspnea in 90% at diagnosis.
  • Bibasilar crackles in 80-90% on exam.
  • Median FVC decline 180-250 ml/year.
  • 6MWD average 250-350 meters at diagnosis.
  • Clubbing in 25-50% of cases.
  • HRCT usual interstitial pneumonia (UIP) pattern in 50-60%.
  • DLCO <40% predicted in 50% advanced IPF.
  • Weight loss in 20-30% patients.
  • Cyanosis in 50% at rest in severe disease.
  • Fatigue reported by 70%.
  • SGRQ score average 45-50 in IPF.
  • Acute exacerbations in 5-10% per year.
  • Chest X-ray abnormal in 90%.
  • BAL lymphocytosis <15% in UIP.
  • GAP index stage I: 6% 1-yr mortality.
  • Velcro rales on auscultation hallmark.
  • Desaturation <88% on 6MWT in 50%.
  • Composite physiologic index >50 in 40%.
  • Orthodeoxia in 20% advanced cases.
  • MRC dyspnea score 3-4 in 60%.
  • HRCT honeycombing in 90% definite UIP.
  • Finger clubbing OR 5.37 for IPF diagnosis.
  • PFT restriction: FVC <80% in 90%.
  • Echo right ventricular systolic pressure >50 mmHg in 30%.
  • LDH elevated in 70% acute exacerbations.
  • KL-6 biomarker elevated in 80%.

Clinical Presentation and Diagnosis Interpretation

This disease often introduces itself with a quiet, stubborn cough and a creeping shortness of breath, but it soon reveals its true nature through the gritty soundtrack of Velcro rales, a landscape of honeycomb lungs, and the body’s quiet surrender in falling oxygen levels and weary exhaustion.

Prevalence and Incidence

  • Idiopathic pulmonary fibrosis (IPF) prevalence in the US is 14-43 per 100,000.
  • Global IPF incidence is 0.6-4.1 per 100,000 person-years.
  • IPF incidence in Europe ranges from 1.3-10.7 per 100,000.
  • UK IPF prevalence increased from 20.1 to 29.8 per 100,000 between 1991-2003.
  • Male predominance in IPF: 1.6-2.4:1 male-to-female ratio.
  • IPF median age at diagnosis is 66 years.
  • IPF accounts for 50% of pulmonary fibrosis cases.
  • Familial IPF represents 2-20% of cases.
  • IPF prevalence in Japan: 5.39 per 100,000.
  • US IPF hospitalizations rose 135% from 2001-2011.
  • IPF mortality in US: 6.8-8.6 per 100,000.
  • IPF prevalence in Olmsted County, MN: 42.7 per 100,000.
  • Global PF prevalence: 13-20 per 100,000.
  • IPF cases in US estimated at 50,000-80,000.
  • Incidence in Australia: 2.3 per 100,000.
  • IPF more common in Caucasians.
  • Annual IPF diagnoses in Europe: ~13,000.
  • Prevalence doubles every decade after 55 years.
  • IPF in US males: 20.25 per 100,000.
  • IPF in US females: 13.51 per 100,000.
  • Pediatric PF incidence: <1 per 100,000.
  • IPF mortality doubled from 1996-2007 in US.
  • Prevalence in Canada: 18.4 per 100,000.
  • IPF incidence in Spain: 2.4 per 100,000.
  • Higher prevalence in rural areas.
  • IPF cases projected to rise 11% by 2025 in US.
  • Familial clustering in 2.2-3.7% of cases.
  • IPF prevalence in Israel: 4.5 per 100,000.
  • Incidence in New Zealand: 4.1 per 100,000.

Prevalence and Incidence Interpretation

Though grimly punctual in its preference for older men and a steady march upward, this stubbornly mysterious disease remains a statistical chameleon, its numbers shifting so wildly across the globe that it’s easier to say it’s always devastating than to pin down exactly how common it is.

Prognosis and Outcomes

  • 80% of IPF patients die within 5 years of diagnosis.
  • Median survival 3-5 years from diagnosis.
  • 1-year mortality 10-15%.
  • GAP stage III: 39% 1-year mortality.
  • Lung transplant 5-year survival 50-60%.
  • Acute exacerbation mortality 50% per event.
  • FVC decline >10% predicts 1-year mortality 40%.
  • DLCO <35% predicted: median survival 1.5 years.
  • DES score >4: poor prognosis.
  • Male gender HR 1.3 for mortality.
  • UIP pattern on HRCT better prognosis than NSIP.
  • Age >65 HR 1.8.
  • Pulmonary hypertension present in 30-50%, worsens survival.
  • 6MWD <250m: 1-year mortality 24%.
  • Hospitalization mortality 20-30%.
  • Nintedanib slows FVC decline by 107 ml/year.
  • Pirfenidone reduces mortality risk 48% at 1 year.
  • Oxygen therapy improves survival by 50%.
  • BMI <21 kg/m2 HR 1.58.
  • Composite GAP index c-statistic 0.80 for mortality.
  • Post-transplant survival median 4.5 years.
  • Annual FVC decline predicts 2-year mortality AUROC 0.73.
  • Serum SP-D >100 ng/ml poor prognosis.
  • Right heart failure in 20%, median survival 8 months.
  • Lung cancer co-occurrence 10-20%, worsens survival.
  • CPI >70: median survival 11 months.
  • 5-year survival post-diagnosis 20-40%.
  • Antifibrotic therapy improves 1-year survival to 95%.
  • GAP stage II: 16% 1-year mortality.
  • Pirfenidone slows FVC decline 110 ml/year.
  • Nintedanib reduces acute exacerbations by 38%.

Prognosis and Outcomes Interpretation

Living with pulmonary fibrosis is a desperate race against time where even our best medicines feel like we're trying to put out a forest fire with a garden hose.

Risk Factors and Etiology

  • Cigarette smoking associated with 48% of IPF cases.
  • Metal dust exposure increases IPF risk by 2.5-fold.
  • Familial history raises risk 3.3-13-fold.
  • Gastroesophageal reflux disease (GERD) in 87% of IPF patients.
  • Smoking history in 63% of IPF cases.
  • Asbestos exposure linked to 5-10% of cases.
  • Genetic mutations (MUC5B) increase risk 3-7 fold.
  • Wood dust exposure OR 1.95 for IPF.
  • Hypersensitivity pneumonitis as precursor in 10-15%.
  • Telomerase mutations in 1-8% familial IPF.
  • Chronic aspiration risk factor in 30-90%.
  • Farming occupation OR 1.3-2.0.
  • TERT gene mutation risk RR 2.7.
  • Obesity BMI>30 reduces IPF risk OR 0.7.
  • Viral infections (EBV, CMV) associated in 20-40%.
  • Stone cutting/polishing OR 2.49.
  • Emphysema co-occurrence in 30-42%.
  • Autoimmune disease overlap in 10-30%.
  • Hairdressing occupation risk increased.
  • SFTPC mutations in familial PF.
  • Socioeconomic status inverse association.
  • Welding fumes OR 1.8.
  • Connective tissue disease in 15% IPF.
  • MUC5B promoter variant in 35% IPF.
  • Pesticide exposure OR 1.6.
  • Raynaud's phenomenon in 15-20%.
  • ABCA3 mutations rare cause.

Risk Factors and Etiology Interpretation

It seems that idiopathic pulmonary fibrosis, far from being truly 'idiopathic', is actually a meticulous bouncer at the lung's exclusive club, letting in a varied guest list of genetic VIPs, blue-collar occupations, smokers, and even the occasional aspirated stomach acid, while curiously turning away the obese.