Health condition · Clinically reviewed
Pulmonary fibrosis, interstitial lung disease and antifibrotic therapy.
Progressive scarring of the lungs — from idiopathic pulmonary fibrosis to hypersensitivity pneumonitis and connective-tissue-related ILD. Antifibrotics slow progression; specialist ILD MDT care matters.
Why trust this guide
- 01
Clinically reviewed
Written by our editorial team and reviewed by a registered UK clinician before publication.
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Sourced from guidance
Checked against NICE, BTS and ERS ILD standards you can see at the end.
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Current for 2026
Reflects UK practice on antifibrotic therapy, ILD MDT referral and transplant pathways.
Key facts
Pulmonary fibrosis at a glance.
The essentials, in plain English — what it is, the common patterns, how it is diagnosed and what modern UK care looks like.
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What it is
A group of interstitial lung diseases (ILD) with progressive scarring — replacing healthy lung with fibrotic tissue.
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Most common form
Idiopathic pulmonary fibrosis (IPF) is the most common progressive fibrotic ILD in adults.
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HRCT pattern
A UIP pattern on high-resolution CT is often diagnostic without a biopsy in the right clinical context.
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Autoimmune and HP
Connective-tissue-related ILD and hypersensitivity pneumonitis are common non-IPF causes.
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Antifibrotic therapy
Nintedanib and pirfenidone slow the decline in lung function in IPF and progressive fibrotic ILD.
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Lung transplant
For selected younger patients with progressive disease, lung transplant is a life-extending option.
Why this guide matters
Modern ILD care has moved on.
Antifibrotics, dedicated ILD MDTs and earlier transplant referral have changed what good pulmonary fibrosis care looks like.
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Getting the ILD subtype right
IPF, autoimmune ILD and hypersensitivity pneumonitis are treated differently — the diagnosis matters.
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Antifibrotics slow decline
Starting nintedanib or pirfenidone early preserves lung function that cannot be regained later.
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Transplant referral is early
For eligible patients, listing before the disease becomes too advanced is what saves lives.
How the diagnosis is made
From first symptoms to a clear ILD diagnosis.
The steps a UK respiratory team will normally follow, in order — so you know what to expect and why.
Phase 1 · History and physiology
Symptoms, exposures, spirometry and DLCO
Phase 2 · Confirming the pattern
HRCT, serology and biopsy if needed
Phase 3 · MDT and baseline
Specialist review and functional baseline
- 01
History and physiology
Symptom, occupational and drug history
Breathlessness and cough — with careful attention to occupational exposure, birds, mould, medications and connective-tissue symptoms.
- 02
History and physiology
Spirometry and DLCO
Restrictive pattern on spirometry with reduced gas transfer (DLCO) is the physiological hallmark.
- 03
Confirming the pattern
HRCT chest
High-resolution CT identifies a UIP or NSIP pattern — a definite UIP appearance is often diagnostic of IPF without biopsy.
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Confirming the pattern
Autoimmune serology
ANA, ENA, RF, anti-CCP, myositis and anti-synthetase panels to identify connective-tissue-related ILD.
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Confirming the pattern
BAL or lung biopsy if uncertain
Bronchoalveolar lavage or surgical/cryobiopsy is used when the HRCT pattern is indeterminate.
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MDT and baseline
ILD multidisciplinary team review
A specialist ILD MDT (respiratory, radiology, pathology, rheumatology) integrates the findings into a working diagnosis.
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MDT and baseline
Baseline 6-minute walk test
Functional walking distance and oxygen desaturation set a baseline to track progression and guide oxygen therapy.
Typical timeline: 6–12 weeks from first tests to a settled ILD MDT diagnosis.
Symptoms
What pulmonary fibrosis actually feels like.
Symptoms usually develop slowly — but a small proportion of people experience sudden acute exacerbations.
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Progressive breathlessness
Slowly worsening breathlessness on exertion — often the earliest and most disabling symptom.
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Dry cough
A persistent, non-productive cough that is often unresponsive to typical cough treatments.
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Finger clubbing
Rounded, bulbous fingertips — a classic physical sign of long-standing fibrotic lung disease.
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Weakness and deconditioning
Reduced exercise tolerance and muscle loss from chronic breathlessness and inactivity.
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Weight loss
Unintentional weight loss and fatigue can develop as the disease progresses.
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Autoimmune features
Joint pain, dry eyes, skin rashes or Raynaud’s may point to connective-tissue-related ILD.
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Post-COVID or occupational
A history of severe COVID pneumonitis or exposure to dusts, moulds or birds is important context.
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Acute IPF exacerbation
Sudden worsening of breathlessness over days — a medical emergency needing urgent respiratory review.
Treatment
How pulmonary fibrosis is treated in the UK.
Antifibrotics for IPF and progressive fibrotic ILD, immunosuppression for autoimmune forms, and supportive therapy that materially improves life.
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Antifibrotic therapy
Nintedanib or pirfenidone — slow the decline in FVC in IPF and progressive fibrotic ILD.
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Immunosuppression
For autoimmune-related ILD — mycophenolate, azathioprine or rituximab under specialist care.
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Long-term oxygen therapy
For chronic resting hypoxaemia meeting LTOT criteria — improves survival and function.
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Pulmonary rehabilitation
Structured exercise and education programme — improves symptoms, walking distance and quality of life.
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Smoking cessation
Essential in every fibrotic lung disease — reduces progression and preserves transplant candidacy.
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Anti-reflux therapy
Proton-pump inhibitors are considered in selected cases where reflux may drive microaspiration.
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Palliative symptom care
Low-dose opioids for breathlessness, cough control and early involvement of specialist palliative teams.
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Lung transplant referral
Early referral for younger, motivated patients with progressive disease and no absolute contraindications.
What this guide is based on
The sources behind every claim on this page.
UK national guidance and specialist society standards, current at the time of last review.
Key references
Guidelines and standards we relied on.
A quiet reminder
This guide is for information, not medical advice.
Your respiratory team or ILD service knows your history and can tell you which parts apply to you. If in doubt, ask for a review.
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NICE. Idiopathic pulmonary fibrosis in adults (NG227).
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British Thoracic Society. Guideline for the diagnosis and management of interstitial lung disease.
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European Respiratory Society / ATS. Clinical practice guidelines on IPF and progressive pulmonary fibrosis.
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Action for Pulmonary Fibrosis. Patient information and support resources.
Red flags
When pulmonary fibrosis becomes an emergency.
Most fibrotic ILD progresses slowly. These are the situations where you should act today.
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Acute IPF exacerbation
Sudden breathlessness over days with new infiltrates on imaging — urgent respiratory admission.
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New pneumothorax
Sudden pleuritic chest pain and worsening breathlessness — call 999 or attend A&E.
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Right heart failure signs
Leg swelling, raised JVP or exertional syncope — suggests pulmonary hypertension complicating ILD.
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Hypoxaemia
Resting or exertional oxygen saturation falling — needs urgent assessment for oxygen therapy.
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Pulmonary hypertension
Disproportionate breathlessness and desaturation — a specialist echo and right-heart catheter may be needed.
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Post-transplant care
Any new fever, breathlessness or rejection concern in a transplant recipient — contact the transplant team immediately.
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Occupational re-exposure
Ongoing exposure to the causative antigen in hypersensitivity pneumonitis will accelerate scarring.
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Rapid decline in DLCO or FVC
A drop of 10% or more in FVC within a year signals progressive disease needing treatment escalation.
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Palliative-stage crisis
Severe uncontrolled breathlessness at end of life — specialist palliative care can transform comfort.
Living with it
A serious condition, but one you can live actively with.
Four things that make the biggest difference day to day — rehabilitation, vaccination, oxygen and community.
A quiet reminder
Ask about antifibrotics and transplant early.
Antifibrotic therapy only helps if it is started. Transplant referral only helps if it happens before the disease is too advanced. Both conversations belong at the first ILD MDT visit, not the last.
- 01 Rehab
Pulmonary rehabilitation works
A structured programme improves walking distance, breathlessness and mood — ask for a referral early.
- 02 Vaccines
Flu, COVID and pneumococcal jabs
Vaccination reduces the risk of infective exacerbations that can drive rapid decline.
- 03 Oxygen
Use ambulatory oxygen well
When prescribed, ambulatory oxygen supports activity and prevents desaturation-driven deconditioning.
- 04 Support
Connect with a patient charity
Action for Pulmonary Fibrosis offers support groups, information and a research community.
Frequently asked
Everything we get asked about pulmonary fibrosis.
Quick answers on IPF, antifibrotics, transplant, inheritance and outlook.
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What is pulmonary fibrosis?
Pulmonary fibrosis is scarring of the lung tissue that stiffens the lungs and reduces oxygen transfer. It is part of a wider group of conditions called interstitial lung diseases (ILDs).
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What is idiopathic pulmonary fibrosis?
IPF is a specific type of progressive fibrotic ILD with no identifiable cause. It typically affects adults over 60 and is diagnosed using HRCT, often showing a UIP pattern.
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Do antifibrotics cure pulmonary fibrosis?
No — nintedanib and pirfenidone do not cure the disease, but they slow the decline in lung function and reduce acute exacerbations for many people.
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When is a lung transplant considered?
For selected younger patients with progressive disease, no significant other organ disease, and the fitness to undergo major surgery and long-term immunosuppression.
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Is pulmonary fibrosis hereditary?
Most cases are not, but familial pulmonary fibrosis exists and genetic testing may be offered where several family members are affected.
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What is the outlook?
The outlook varies widely depending on the type, cause and rate of progression. Antifibrotics, transplant referral and specialist ILD MDT care have meaningfully improved outcomes.
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