Fibrosis and interstitial lung disease

Which part of the lung is affected?

The lung has millions of tiny air sacs whose thin walls lie against capillaries. Oxygen reaches the blood through this wall. In interstitial lung disease, the tissue around these sacs becomes thick and stiff. When the change is lasting and leaves scarring, it is called fibrosis, and breathing slowly becomes harder.

Interstitial lung disease is not one disease but many. Idiopathic pulmonary fibrosis, meaning fibrosis with no known cause, is the most common type and mostly affects people over 70. Some other types are linked to dust, mould, birds or medicines.

What are the symptoms?

• Breathlessness that slowly grows, first on effort and later with ordinary tasks
• A persistent dry cough
• Marked tiredness
• Rounded fingertips with curved nails

These symptoms arrive slowly, and many people put them down to age or being unfit. If your cough has passed three weeks, or you run out of breath sooner in your usual activities, do not wait.

How is the type found?

The history matters a great deal in these diseases. Think about these before your visit:

• Past jobs and contact with metal or wood dust or asbestos
• Birds, mould or damp at home and at work
• Every medicine you have taken
• A similar illness in close relatives
• Cigarettes and water pipe

Spirometry, a chest X-ray and a CT usually follow, and blood tests are sometimes needed. If the cause is still unclear, a bronchoscopy or a lung biopsy may be suggested.

What can treatment do?

To be plain: treatment does not reverse scarring, and idiopathic pulmonary fibrosis has no cure at present. Treatment is still worthwhile:

• In idiopathic pulmonary fibrosis, antifibrotic medicines can slow progression. They are monitored with blood tests.
• If an environmental cause is found, avoiding it is the first step.
• Oxygen, when needed, eases breathlessness and helps you stay active.
• Pulmonary rehabilitation brings exercise and education together.
• Recommended vaccines and stopping smoking help prevent infections and worsening.
• In the few cases that keep progressing, a lung transplant is considered.

How fast it progresses varies widely between people, and some have few symptoms for years. That is why regular follow-up with spirometry and oxygen checks matters.

What about cell therapy?

Many fibrosis patients ask about cell therapy. This field is still at the research stage. Read the honest account on the cell therapy page, and do not set your medicines aside in the hope of it.