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Health condition

Interstitial lung disease, from IPF to CTD-ILD, and how UK ILD centres treat it.

Over 200 conditions - one careful pathway. HRCT, a specialist multidisciplinary team, antifibrotics, rehab, oxygen and, for the right patient, transplant. This is our companion guide to our main ILD overview.

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A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Editorial team

    Written by our editorial team from the UK sources listed on each page.

  • 02

    Sourced from guidance

    Checked against NICE, BTS, ATS/ERS/JRS/ALAT and NHS specialist ILD commissioning standards.

  • 03

    Current for 2026

    Reflects modern UK practice - antifibrotics, MDT-led diagnosis and specialist commissioned ILD centres.

Key facts

ILD at a glance.

A quick orientation to a large family of conditions - the subtypes, the symptoms, and the modern UK pathway. Deeper detail lives in our main ILD overview.

  • What it is

    A family of over 200 diseases that scar or inflame the lung interstitium - the delicate tissue that surrounds and supports the air sacs.

  • Main subtypes

    IPF, NSIP, COP, AIP, DIP and RB-ILD, LIP, connective tissue disease ILD, hypersensitivity pneumonitis, sarcoidosis, occupational and drug or radiation-induced ILD.

  • Cardinal symptoms

    Progressive breathlessness on exertion, dry cough, fatigue - often with fine end-inspiratory crackles and, in some, clubbing.

  • Diagnostic backbone

    High-resolution CT of the chest, full lung function with gas transfer and a specialist ILD multidisciplinary team discussion.

  • Antifibrotics

    Nintedanib and pirfenidone slow decline in IPF and in progressive pulmonary fibrosis of other causes - prescribed through specialist ILD centres.

  • Beyond drugs

    Pulmonary rehabilitation, oxygen assessment, vaccination, palliative symptom care and, in selected patients, lung transplant.

Why this guide matters

One family, many faces.

IPF is the best-known ILD - but NSIP, COP, AIP, DIP or RB-ILD, LIP, hypersensitivity pneumonitis, sarcoidosis, connective tissue disease ILDs, occupational lung disease and drug or radiation-induced fibrosis all belong here. Getting the label right changes the treatment.

  • HRCT is the pivot

    A high-resolution CT is the single most important test - UIP, NSIP, OP, HP and sarcoid all have recognisable patterns that guide everything after.

  • A specialist MDT should decide

    NHS England commissions ILD as a specialised service - the diagnosis is agreed by a dedicated multidisciplinary team, not a single clinician.

  • Antifibrotics changed the outlook

    Nintedanib and pirfenidone slow decline in IPF and in progressive pulmonary fibrosis - alongside rehab, oxygen and, in selected patients, transplant.

How the diagnosis is made

From breathless on the stairs to a specialist plan.

The steps a UK respiratory team will normally follow, in order - so you know what to expect at each stage. A lung function test and a private CT scan often anchor the workup.

  1. 01

    Assessing

    History and exposures

    A careful timeline - breathlessness, cough, occupational dusts, birds, mould, hot tubs, drugs, radiotherapy and family history of pulmonary fibrosis.

  2. 02

    Assessing

    Examination and oximetry

    Fine end-inspiratory crackles, clubbing, resting and exertional oxygen saturation and a search for connective tissue disease signs.

  3. 03

    Assessing

    Bloods and autoimmune screen

    FBC, renal, liver, ANA, ENA, RF, anti-CCP, myositis panel and ANCA - to look for CTD-ILD, vasculitis and hypersensitivity clues.

  4. 04

    Confirming

    High-resolution CT (HRCT)

    The single most important test - patterns such as UIP, NSIP, OP, DIP and HP guide the differential and the need for biopsy.

  5. 05

    Confirming

    Lung function and 6-minute walk

    Spirometry, lung volumes and transfer factor (TLCO) grade severity; a 6-minute walk shows desaturation and functional impact.

  6. 06

    Confirming

    Bronchoscopy or biopsy if needed

    Bronchoalveolar lavage or transbronchial cryobiopsy in selected cases when HRCT and clinical data leave the diagnosis unclear.

  7. 07

    Deciding

    Specialist ILD MDT

    A dedicated multidisciplinary team - ILD physician, thoracic radiologist, pathologist and rheumatologist - agrees the diagnosis and plan.

Typical timeline: from GP concern to an MDT-agreed plan in a matter of weeks in a well-run pathway.

Symptoms

What ILD actually feels like.

A slow crescendo of breathlessness and cough is the usual story - punctuated by the extra clues (crackles, clubbing, CTD features) that shape the differential.

  • Breathlessness on exertion

    The commonest presenting symptom - often creeping up over months, first noticed climbing stairs or hills.

  • Persistent dry cough

    A hacking, non-productive cough that outlasts every infection and is often the most intrusive symptom.

  • Fatigue and reduced stamina

    Systemic tiredness, poor sleep and reduced exercise tolerance - often out of proportion to spirometry.

  • Fine end-inspiratory crackles

    Velcro-like crackles at the lung bases - a clinical hallmark of fibrotic ILD, particularly IPF.

  • Finger clubbing

    Present in a significant minority - especially in IPF, asbestosis and some CTD-ILDs.

  • Extra-pulmonary CTD features

    Raynaud phenomenon, sclerodactyly, arthritis, dry eyes and mouth, rashes or myositis - clues to a rheumatological driver.

  • Hypoxia and desaturation

    Falling oxygen saturation on walking is common in progressive ILD - and a trigger for oxygen assessment.

  • Red flag - acute deterioration

    Sudden worsening of breathlessness over days may be an acute exacerbation, infection or pulmonary embolism - a same-day emergency.

Treatment

How ILD is treated in the UK.

Antifibrotics and immunosuppression when the pattern calls for them - always alongside pulmonary rehabilitation, oxygen assessment, vaccination and, in selected patients, lung transplant.

  • Antifibrotics (nintedanib, pirfenidone)

    Slow lung function decline in IPF and in progressive pulmonary fibrosis of other causes - prescribed and monitored through NHS commissioned ILD centres.

  • Immunosuppression

    Steroids with mycophenolate, azathioprine or rituximab for NSIP, COP, HP and CTD-ILD - chosen by the specialist ILD team.

  • Antigen avoidance

    The cornerstone of hypersensitivity pneumonitis care - removing birds, mould, feathers or occupational exposures often halts progression.

  • Pulmonary rehabilitation

    A supervised exercise, education and breathing programme that reliably improves breathlessness, stamina and quality of life.

  • Long-term oxygen therapy

    For patients with resting or exertional hypoxia - prescribed after formal assessment and titrated to keep saturations safe.

  • Vaccination and infection prevention

    Annual influenza, pneumococcal and COVID vaccines - lower respiratory infections are a major cause of acute exacerbations.

  • Palliative symptom care

    Low-dose opioids for refractory breathlessness, cough control and early involvement of supportive care teams.

  • Lung transplantation

    The only intervention that changes long-term prognosis in advanced disease - referral early rather than late is the modern approach.

What this guide is based on

The sources behind every claim on this page.

UK and international respiratory 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 GP and ILD team know your imaging, lung function and history and can tell you which parts apply to you. If in doubt, get seen.

  • NICE. Idiopathic pulmonary fibrosis in adults: diagnosis and management (NG216).

  • ATS/ERS/JRS/ALAT. Diagnosis of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis clinical practice guidelines.

  • British Thoracic Society (BTS). Guidelines on interstitial lung disease and pulmonary rehabilitation.

  • NHS England. Specialised respiratory service specifications for interstitial lung disease.

Red flags

When ILD needs urgent attention.

Most ILD is managed in a slow, planned way - but a handful of situations mean a same-day call rather than a next-clinic conversation.

  • Acute exacerbation of ILD

    Rapid worsening of breathlessness with new HRCT infiltrates over days - carries high mortality and needs urgent ILD centre input.

  • Suspected pulmonary embolism

    Sudden pleuritic pain, drop in saturations or unexplained tachycardia - needs same-day assessment with CTPA.

  • Superimposed infection

    Fever, productive cough or new consolidation on imaging - a common trigger for deterioration in fibrotic lung disease.

  • Pneumothorax

    Sudden one-sided chest pain and breathlessness - fibrotic lungs are prone to spontaneous pneumothorax.

  • Rapidly progressive fibrosis

    Fall in FVC of 10% or TLCO of 15% over 12 months signals progressive pulmonary fibrosis and often prompts antifibrotic therapy.

  • New pulmonary hypertension

    Marked exertional desaturation, syncope or right heart signs - warrants echocardiography and expert review.

  • Suicidality or severe low mood

    Living with progressive breathlessness is hard - low mood, hopelessness or suicidal thoughts need urgent GP or crisis support.

  • Anti-MDA5 or anti-Jo-1 antibody

    Myositis-associated ILD can progress fast - rheumatology and ILD teams need to move together.

  • Drug-induced or radiation ILD

    Amiodarone, methotrexate, nitrofurantoin, bleomycin, checkpoint inhibitors and chest radiotherapy - stopping the drug and steroid cover may be needed.

Living with it

A long condition, with real levers.

Four habits and decisions that reliably improve day-to-day life with ILD - and one signal that it is time to ask for more from your team.

A quiet reminder

Being seen early beats being seen late.

If you are not yet under a specialist ILD centre, or if antifibrotics or transplant have never been discussed, that is a fair question to bring to your next appointment.

  1. 01 Pace

    Pulmonary rehab first

    A supervised programme is one of the single most effective interventions - it retrains breathing, builds stamina and reduces breathlessness.

  2. 02 Protect

    Vaccines and infection care

    Flu, pneumococcal and COVID vaccines every autumn - and a low threshold for seeking help with any chest infection.

  3. 03 Movement

    Keep moving, gently

    Regular walking, cycling or swimming within your limits preserves function - stopping activity makes breathlessness worse, not better.

  4. 04 Escalate

    Ask about specialist review

    If breathlessness is progressing, if you have not been to an ILD centre, or if you have never discussed transplant - ask.

Frequently asked

Everything we get asked about ILD.

Straight answers on subtypes, HRCT, antifibrotics, pulmonary rehab, oxygen and transplant.

  • What exactly is interstitial lung disease?

    ILD is an umbrella term for over 200 conditions that inflame or scar the interstitium - the delicate tissue around the alveoli. Some are idiopathic (IPF, NSIP, COP, AIP), some are driven by connective tissue disease, hypersensitivity, occupational exposure, drugs or radiotherapy, and others such as sarcoidosis have their own family of patterns.

  • How is it different from idiopathic pulmonary fibrosis?

    IPF is one specific type of ILD - a progressive fibrotic disease with a usual interstitial pneumonia (UIP) pattern on HRCT. All IPF is ILD, but not all ILD is IPF. NSIP, COP, HP, sarcoidosis and CTD-ILDs behave very differently and are often more treatable.

  • Why do I need a specialist ILD centre?

    Diagnosis relies on a multidisciplinary team - an ILD physician, thoracic radiologist, pathologist and rheumatologist - reviewing your history, HRCT, lung function and any biopsy together. NHS England commissions this as a specialised service, and antifibrotic drugs are prescribed through these centres.

  • What are antifibrotics and who gets them?

    Nintedanib and pirfenidone are oral drugs that slow the rate of lung function decline. They are licensed for IPF and, more recently, for progressive pulmonary fibrosis of other causes - including some CTD-ILDs, chronic HP and unclassifiable ILD. They do not reverse scarring but can meaningfully change the trajectory.

  • Should I be thinking about lung transplant?

    For fit patients with advancing disease, transplant is the only intervention that changes long-term prognosis. Modern practice is to discuss it early, well before you are severely unwell - so referral to a UK transplant centre for assessment is worth raising with your ILD team.

  • What about pulmonary rehabilitation and oxygen?

    Pulmonary rehabilitation is one of the most evidence-based interventions in ILD - it reliably improves breathlessness, stamina and quality of life. If you are hypoxic at rest or on exertion, a formal oxygen assessment through your respiratory team can determine whether long-term or ambulatory oxygen would help.

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