Interstitial means “at intervals” in Latin. In medical terms, this word is usually used to denote interstitial lung disease. Interstitial lung disease (ILD) describes a large group of conditions that cause swelling and scarring of lung tissues. The scarring leads to stiffness in the lungs, making it difficult to breathe. ILD can affect other parts of the lungs too, such as the airways, lung lining and blood vessels. Once lung damage occurs, it is generally irreversible. ILD can be a difficult disease to treat, sometimes progressing slowly and, in some patients, very quickly. The common symptoms may include
- Shortness of breath
- Labored breathing
- Recurrent dry, unproductive cough
- Fatigue and weakness
- Nails that bulge or thicken
- Widening and rounding of fingertips (clubbing)
- Rapid, shallow breathing
- Muscle and joint pain
- Loss of appetite
- Unexplained weight loss
- Discomfort in the chest
- Bleeding (hemorrhage) in the lungs
What are the causes and risk factors of interstitial lung disease?
- Lung diseases or infections
- Long-term exposure to irritants, such as cigarette smoke, dust, gasses or fumes from industry or agriculture
- Autoimmune diseases
- Certain medicines, such as
- Genetic disorders
- Cancer treatments, such as chest radiation and chemotherapy can also cause damage lung tissue
- Occupational exposure to heavy metals, allergens (beryllium) or fibers
There are many causes for inflammation and scarring (fibrosis) of lung tissue. Underlying connective tissue or autoimmune diseases, such as rheumatoid arthritis, scleroderma or myositis can cause swelling of lung tissue. Some forms of interstitial lung diseases could be genetic. Inhalation of certain proteins, especially from feathers and molds, can also cause swelling. If swelling is present in the lung for a long time, scarring of the lung can occur. One of the most common types of interstitial lung disease is idiopathic pulmonary fibrosis (IPF), which is associated with aging and results in scarring of the lung, rather than swelling. Often the occupational lung diseases, such as the coal worker’s pneumoconiosis, asbestosis and bagasse exposure can also manifest as ILD.
What are the treatment options for interstitial lung disease?
The treatment options may include an individualized treatment plan based on each patient’s overall health, specific symptoms and other health conditions. Treatment plans can include one or more options.
Medications to treat ILD and its symptoms
- Esbriet (pirfenidone) and Ofev (nintedanib) to slow the progression of the disease.
- Tracleer (bosentan) is a drug that acts on the inner lining of the blood vessels of the lung. This medication is used in the treatment of pulmonary artery hypertension, which often accompanied the ILD. Sildenafil may also be used for this purpose.
- Prednisone and other immunosuppressant medications to reduce the amount of inflammation in the lungs.
- Anti-acid medications to treat gastroesophageal reflux disease (GERD), which frequently occurs in people with pulmonary fibrosis.
- Nose sprays and inhalers to help treat cough.
- Nonsurgical therapies include oxygen therapy (which can ease breathing) reduce the risk of complications from low blood oxygen levels and prevent high blood pressure in the lungs (pulmonary hypertension).
- Pulmonary rehabilitation is a long-term, outpatient program with specialized therapists. Pulmonary rehabilitation can improve lung function and overall health and is always recommended after a lung transplant. A pulmonary rehabilitation program may include
- For people with advanced pulmonary fibrosis and severe lung damage, a single or double lung transplant can improve the ability to breathe and exercise.
The prognosis of ILD may vary, depending on the subtype of ILD. Regularly screening high-risk patients help diagnose the disease early, and early interventions could improve the treatment outcome as well.
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Antoine M, Mlika M. Interstitial Lung Disease. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021. https://www.ncbi.nlm.nih.gov/books/NBK541084/