Interstitial lung disease overview

Overview

Interstitial lung disease (ILD) is a group of lung conditions that cause scarring, called fibrosis, in the tissue between the air sacs of your lungs. This scarring makes your lungs stiff and less able to take in oxygen, which can lead to shortness of breath and a dry cough.

ILD is relatively rare compared to other lung diseases like asthma or COPD. However, it is more common in people over 50 and is becoming better recognized.

It can affect people of all ages, but it is most often diagnosed in middle-aged and older adults. Men are slightly more likely to get it than women. Some types of ILD are more common in people with a family history, or in those who have been exposed to certain environmental triggers.

Symptoms

Common symptoms

• Shortness of breath, especially when active
• A dry, hacking cough that doesn't go away
• Fatigue or feeling very tired
• Unintended weight loss
• Clubbing – where the tips of the fingers become wider and rounder

Symptoms in children

• In children, ILD is rare but can cause similar symptoms: trouble breathing, cough, poor growth, and low energy.

Symptoms in older adults

• Older adults may notice that everyday activities like walking or climbing stairs become harder. They can also feel more breathless and tired. Sometimes symptoms are mistaken for normal aging.

Causes

Main causes

• Autoimmune diseases like rheumatoid arthritis or scleroderma, where the immune system attacks the lungs
• Exposure to harmful substances at work or in the environment, such as asbestos, silica dust, coal dust, or mold
• Certain medications, including some chemotherapy drugs, heart medicines, and antibiotics
• Radiation therapy to the chest area
• In many cases, the cause is unknown (this is called idiopathic ILD)

Risk factors

• Being middle-aged or older (over 50)
• Smoking or having a history of smoking
• A family history of ILD or certain genetic factors
• Working in industries with dust or chemical exposure (farming, mining, construction, manufacturing)
• Having an autoimmune disease like rheumatoid arthritis, lupus, or sarcoidosis

When to see a doctor

See a doctor urgently if:

• If you have sudden, severe shortness of breath, especially with chest pain or coughing up blood
• If your lips or skin turn blue
• If you are unable to walk a few steps without gasping for air

Book a routine appointment if:

• If you have a persistent cough that lasts more than a few weeks
• If you feel breathless when doing things you used to do easily
• If you have unexplained fatigue, weight loss, or finger clubbing

Diagnosis

Your doctor will start by asking about your symptoms, medical history, and any exposures to dust or chemicals. They will listen to your lungs and may order tests to see how your lungs are working and to look for scarring.

Tests that may be done

• Chest X-ray to look for any visible changes in the lungs
• High-resolution CT scan (HRCT) to get detailed images of the lung tissue
• Pulmonary function tests (PFTs) to measure how well you breathe
• Blood tests to check for autoimmune conditions or infections
• Bronchoscopy with biopsy – a thin tube is passed into the lungs to take a small sample of tissue for examination

What to expect at your appointment

Diagnosis can take time and may involve several visits to a lung specialist (pulmonologist). You might need more than one test. The goal is to find out the type of ILD you have and how advanced it is, which helps guide treatment.

Treatment

Treatment for ILD depends on the type and severity. The main goals are to slow the scarring, manage symptoms, and improve your quality of life. Many people are treated by a team of specialists including lung doctors, physical therapists, and dietitians.

Self-care at home

• Quit smoking if you smoke – this is one of the most important steps
• Avoid exposure to dust, chemicals, and fumes
• Get vaccinated against flu and pneumonia (ask your doctor)
• Stay active with gentle exercise like walking – but listen to your body
• Eat a balanced diet to maintain a healthy weight
• Use oxygen therapy if prescribed – it can help you be more active

Medical treatments

Doctors may prescribe medications that help reduce inflammation or slow fibrosis. These may include antifibrotic drugs or immune-suppressing medicines. Your doctor might also recommend oxygen therapy, pulmonary rehabilitation (a program of exercise and education), or in some cases, a lung transplant if the disease is advanced.

When is surgery considered?

Lung transplant is an option for some people with very severe ILD who are otherwise healthy enough for major surgery. It is not a common treatment but can improve survival and quality of life for the right candidate.

Living with this condition

Living with ILD means managing shortness of breath and fatigue. You might need to plan your day to save energy. Using a fan, pacing yourself, and doing breathing exercises can help. Many people use oxygen at home or while moving around.

Lifestyle tips

• Quit smoking and avoid second-hand smoke
• Stay up to date with flu and pneumonia vaccines
• Avoid lung irritants like strong fumes, perfumes, and dust
• Get enough rest and sleep
• Manage stress with relaxation techniques like deep breathing or gentle yoga

Diet and exercise

A balanced diet helps you maintain a healthy weight – being too heavy can make breathing harder, and being too underweight can weaken your muscles. Gentle exercise like walking or stretching, as tolerated, helps keep your body strong. Pulmonary rehabilitation programs are designed for people with lung disease and can be very helpful.

Mental health and emotional wellbeing

Having a chronic lung condition can be stressful and may lead to feelings of anxiety or depression. It's normal to feel worried. Talking to a counselor, joining a support group, or simply sharing your feelings with loved ones can make a big difference.

Prevention

Not all cases of ILD can be prevented, especially those linked to genetics or autoimmune diseases. However, you can reduce your risk by avoiding smoking and limiting exposure to lung-damaging substances at work and home.

Vaccines

Vaccines cannot prevent ILD itself, but getting the flu shot and pneumonia vaccine can help prevent infections that might worsen your lung condition. Ask your doctor which vaccines are right for you.

Screening programmes

There is no routine screening test for ILD in people without symptoms. If you have a family history of ILD or work in a high-risk job, talk to your doctor about whether monitoring is recommended.

Complications

If left untreated

• Progressive scarring that makes it harder to breathe over time
• Low oxygen levels in the blood, which can affect your heart and other organs
• Pulmonary hypertension (high blood pressure in the arteries of the lungs)
• Increased risk of lung infections and acute exacerbations (sudden worsening)
• Reduced ability to perform daily activities and decreased quality of life

Long-term outlook

The outlook for ILD varies greatly depending on the type and how early it is found. Some forms respond well to treatment and remain stable for many years. For others, the disease may progress more quickly. However, new treatments are helping people live longer and better with ILD. With good medical care, support, and self-management, many people lead fulfilling lives.

Find support

External links open third-party websites. Ruqelo Health is not responsible for external content. Listing an organisation does not imply endorsement.