What are interstitial lung diseases (ILDs)?

Interstitial lung diseases (ILDs) are a broad group of disorders that lead to inflammation or scarring in the lungs. This inflammation and scarring make it difficult to get enough oxygen. Scarring in the lungs is known as pulmonary fibrosis [1]. In these conditions, tissues located in or around the air sacs and airways are damaged, affecting the lungs' ability to perform their primary function of gas exchange [3].

The interstitial space in the lungs—the area between the air sacs and the small surrounding blood vessels—becomes thick and stiff. This stiffness hinders the movement of oxygen from the lungs into the bloodstream, and the movement of carbon dioxide from the bloodstream into the lungs [3]. Interstitial lung diseases can range from mild to severe, and in some cases, they can be life-threatening [3].

Causes of Interstitial Lung Diseases

There are many causes of ILDs, including exposure to certain environmental substances, autoimmune diseases, and sometimes they can be hereditary [1]. Notable known causes include:

• Exposure to chemicals and dust: Inhaling coal dust (black lung disease), farm dust (farmer's lung), asbestos fibers (asbestosis), silica dust (silicosis), or iron and welding fumes (siderosis) [1].
• Autoimmune diseases: Such as lupus, rheumatoid arthritis, and scleroderma [2].
• Genetics: Certain genetic mutations that affect lung development or surfactant production can cause ILDs [2].
• Medications: Certain drugs such as chemotherapy, radiation, immunosuppressants, and some antibiotics and heart medications [2].
• Infections: Certain bacterial or viral infections can increase the risk of developing the condition [2].
• Unknown causes: In many cases, there is no clear cause for ILD; the most common type with an unknown cause is idiopathic pulmonary fibrosis (IPF) [3].

Common Symptoms

The main symptoms of ILDs include shortness of breath, a dry cough, chest discomfort, and severe fatigue [3]. In children, symptoms may include shortness of breath, rapid breathing, a chronic cough, and growth and development issues. Signs such as skin discoloration (cyanosis) or clubbing (widening and rounding of the fingertips or toes with nail curving) may appear [2].

Diagnosis and Treatment

Diagnosis depends on the patient's medical and family history, a physical examination, lung function test results, blood tests, and genetic testing [3]. There is no complete cure for ILDs, as existing scarring in the lungs cannot be repaired [3]. However, early treatment can help slow or stop lung damage and improve lung function [3].

Treatment options include medication, oxygen therapy, and in severe cases, a lung transplant may be necessary [1]. The specific treatment depends on the type of disease and the severity of symptoms [3]. Doctors may recommend avoiding environmental substances that trigger the condition [3]. For more information on pulmonary fibrosis (Arabic).

Practical Steps and When to Seek Emergency Help

It is important for those with ILDs to adhere to the recommended treatment plan and follow up regularly with their healthcare team. Ensure that you receive recommended vaccinations, such as influenza and pneumonia vaccines, to help prevent infections that could exacerbate symptoms [2]. It is also advised to avoid exposure to secondhand smoke and to stay indoors when air quality is poor [2].

Emergency care should be sought immediately if the patient experiences severe difficulty breathing, if a blue color appears on the skin, lips, or fingernails, or if the patient becomes confused, extremely drowsy, or loses consciousness [2].

Medical references

• Interstitial Lung Diseases
• Interstitial Lung Diseases What Are Interstitial Lung Diseases?
• Interstitial Lung Diseases Childhood Interstitial Lung Disease