Chronic Fibrosing Interstitial Lung Disease
Chronic fibrosing interstitial lung disease (ILD) is a group of conditions in which scarring thickens lung tissue, causing dry cough and progressive breathlessness.
Key takeaways
- It is a pattern, not one diagnosis; autoimmune disease, exposures, medicines and idiopathic pulmonary fibrosis are possible causes.
- High-resolution CT and serial lung-function tests help define the subtype and whether fibrosis is progressing.
- Anti-inflammatory and antifibrotic treatments have different indications; suppressing immunity can harm some fibrotic diseases.
The listings below do not establish the ILD subtype; specialist multidisciplinary assessment should guide treatment.
Establishing cause and progression
Evaluation combines exposure and medicine history, autoimmune symptoms, examination, CT pattern and lung-function trends. Blood tests, bronchoscopy or lung biopsy are used selectively when they can change diagnosis. Oxygen testing during exertion can reveal impairment not present at rest.
Managing disease and function
Remove a causative exposure where possible. Immunomodulation may help inflammatory autoimmune ILD, while nintedanib or pirfenidone may slow decline in specified progressive fibrosing conditions. Pulmonary rehabilitation, vaccination, oxygen when indicated and transplant assessment address function and complications.
When to seek urgent care
Seek urgent care for sudden or rapidly worsening breathlessness, blue or grey lips, chest pain, confusion, coughing blood or a substantial new drop in oxygen saturation.