Disease Directory Idiopathic Pulmonary Fibrosis
Respiratory

Idiopathic Pulmonary Fibrosis

Also known as: IPF, cryptogenic fibrosing alveolitis, UIP pattern

Prevalence

Approximately 3–9 per 100,000

Onset

Adult (typically over age 50)

Type

Rare progressive fibrosing interstitial lung disease

About Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is a chronic, progressive, and ultimately fatal fibrosing interstitial lung disease of unknown cause, occurring primarily in older adults. Histologically and radiologically characterised by a usual interstitial pneumonia pattern, IPF leads to progressive scarring of lung tissue, worsening dyspnoea, and declining lung function. Two antifibrotic agents, nintedanib and pirfenidone, slow progression but do not reverse the disease.

Common Clinical Features

Progressive dyspnoea on exertion Persistent dry cough Bibasilar fine crackles on auscultation Clubbing of fingers and toes Fatigue Honeycombing with traction bronchiectasis on HRCT Hypoxaemia and declining DLCO

Clinical Trial Eligibility Tips

What to know before applying to Idiopathic Pulmonary Fibrosis trials.

Most trials require a confirmed UIP pattern on HRCT or surgical lung biopsy and a minimum FVC (commonly ≥45%) and DLCO (commonly ≥30%); have recent PFTs available.

Current use of nintedanib or pirfenidone may be a criterion or exclusion depending on the study; clarify whether the trial is add-on or treatment-naive.

Acute exacerbations within a defined period (typically 6 months) frequently trigger exclusion; apply during a clinically stable phase.

Patient Resources

Patient Organization

Pulmonary Fibrosis Foundation

Visit website ↗

Natural History Registry

PFF Patient Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:2032)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Idiopathic Pulmonary Fibrosis trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Idiopathic Pulmonary Fibrosis. Updated daily.

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