Idiopathic pulmonary fibrosis

Also called IPF, Pulmonary fibrosis, Interstitial lung disease

Scarring of the lungs with no known cause. Velcro-like crackles and finger clubbing are the clues, and it is usually diagnosed as something else first.

What it is

Progressive scarring of the lung tissue, with no identified cause, mainly affecting people over 60 and more men than women.

The scarring stiffens the lungs and reduces oxygen transfer, causing breathlessness that worsens over time. It is not reversible, and average survival from diagnosis is around three to five years, though the course varies considerably and some people remain stable for long periods.

It is routinely misdiagnosed at first as COPD, asthma, heart failure or simply age, and the average delay to diagnosis is well over a year. Two clinical signs point to it: fine crackles at the lung bases that sound like Velcro being pulled apart, and clubbing of the fingertips.

Antifibrotic drugs, pirfenidone and nintedanib, slow the decline and are available on the NHS through specialist centres, with criteria attached. Getting to a specialist interstitial lung disease service is what unlocks them, along with trials, oxygen assessment and transplant consideration.

Pulmonary rehabilitation improves breathlessness and exercise capacity and is under-used.

Acute exacerbations, sudden worsening over days, carry a high mortality and need urgent assessment rather than being managed at home.

Signs you might notice

Breathlessness on exertion, gradually worsening over months.

A persistent dry cough, often the most troublesome symptom and unresponsive to the usual remedies.

Fatigue, and unintentional weight loss.

Clubbing of the fingers: nails becoming curved and the fingertips broadening.

Fine crackles at the lung bases, heard with a stethoscope.

Reduced oxygen levels on exertion, sometimes with normal readings at rest, which is why walking tests matter.

Urgent: a sudden worsening of breathlessness over days, or with fever or coloured sputum. That may be an exacerbation or infection and needs same-day assessment.

Anybody with unexplained breathlessness and a dry cough over 60 should have their chest listened to and a chest X-ray, and normal spirometry does not exclude it.

How it can affect day-to-day life

The cough is often what people mind most: relentless, dry, socially difficult, and poorly treated. It deserves specific attention rather than being treated as a side issue.

Oxygen is prescribed for low levels on exertion as well as at rest, and ambulatory oxygen assessment is frequently not offered.

Breathlessness generates fear, and fear worsens breathlessness. Breathing control techniques, handheld fans and pacing genuinely help and are taught in pulmonary rehabilitation.

The prognosis conversation is often avoided, which leaves people unable to plan. Early advance care planning and early palliative involvement improve symptom control and are not the same as giving up.

Flu, COVID and pneumococcal vaccinations matter more here than for most people, because infections trigger exacerbations.

Carers carry a heavy load, and this progresses faster than most long-term conditions.

Supporting someone well

Ask for referral to a specialist interstitial lung disease service. That is where antifibrotic drugs, trials and transplant assessment happen.

Ask about antifibrotic treatment specifically.

Do pulmonary rehabilitation; it works and it is under-referred.

Ask for ambulatory oxygen assessment, with a walking test, not just a resting reading.

Ask for the cough to be treated in its own right.

Keep vaccinations up to date.

Learn breathing control and use a handheld fan; both reduce the sensation of breathlessness.

Get sudden worsening assessed the same day.

Start advance care planning early, and ask for palliative care alongside active treatment.

Where to get help

A GP practice for a chest X-ray and referral; a specialist interstitial lung disease centre for management.

Same-day assessment for sudden worsening.

Action for Pulmonary Fibrosis run a support line and are the specialist charity.

Asthma + Lung UK helpline: 0300 222 5800.