About rare interstitial lung diseases (rare ILD)

What are rare interstitial lung diseases? 

Rare interstitial lung diseases (rare ILDs) are a group of conditions that cause scarring and inflammation in the lung tissue. They affect the interstitium — the delicate network of tissue that supports the air sacs in the lungs — and make it harder for oxygen to pass into the blood to move around the body.  

Some rare ILDs are linked to other diseases or harmful things in the environment. Other rare ILDs are genetic, are triggered by the immune system, or have no known cause.

Rare ILDs can affect children and adults but many forms are only recognised in adulthood. This means that symptoms in younger people can go undiagnosed for years. The term “childhood interstitial lung disease” or chILD is used to refer to more than 200 rare lung conditions that affect children. 

What are the challenges of living with a rare interstitial lung disease? 

The way that an ILD presents — and how it progresses — can vary widely from person to person. Common symptoms include breathlessness, persistent dry cough and fatigue. Because these symptoms overlap with other respiratory conditions, it can take time for a patient to receive an accurate diagnosis. Over time, scarring can cause the patient’s lung function to decline and make it increasingly difficult for them to breathe. Some rare ILDs also cause inflammation in other organs, such as the skin or joints. 

Many people living with rare ILDs experience significant delays in diagnosis and uncertainty about how their condition will develop over time. Living with a rare disease can affect mental health and wellbeing. For example, when a condition has a genetic cause, people can feel worried about how their family may be affected. 

What care and support is available? 

There are currently no cures for rare ILDs, but early diagnosis and specialist care can help slow disease progression. Depending on the diagnosis, treatment may include corticosteroids, immunosuppressive medications or antifibrotic drugs. Patients might also receive oxygen therapy, pulmonary rehabilitation and, in severe cases, lung transplantation.  

Remember, every person is different. Always ask your doctor or another trained health professional if you have concerns about your health.

Learn how our Centre is working to improve the diagnosis and treatment of rare ILD >