What is ILD?

Interstitial lung disease is another term for pulmonary fibrosis, or “scarring” and “inflammation” of the interstitium (the tissue that surrounds the lung’s air sacs, blood vessels and airways). Analogy of grapes is good for understanding of nature of ILD lungs. Normal lungs are like grapes and ILD lungs are like raisins. ILD lungs are also shrunken and hard as  raisins.

Interstitial lung disease describes a large group of disorders, most of which cause progressive scarring of lung tissue. The scarring associated with interstitial lung disease eventually affects your ability to breathe and get enough oxygen into your bloodstream

Interstitial lung disease can be caused by long-term exposure to hazardous materials, such as asbestos. Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. In some cases, however, the causes remain unknown

Once lung scarring occurs, it’s generally irreversible. Medications may slow the damage of interstitial lung disease, but many people never regain full use of their lungs. Lung transplant is an option for some people who have interstitial lung disease

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interstitial lung disease
What are symptoms of ILD?

Most people with ILD have symptoms that make breathing difficult, including:

  • Shortness of breath
  • Coughing, typically nonproductive
  • Decreased exercise tolerance
  • Fatigue
  • Weight loss
What causes interstitial lung disease?

There are more than 200 causes of ILD. Types of ILD include:

ILD related to another health disorder: Some people develop ILD as a result of having an autoimmune disease (the immune system harms the body). Examples of autoimmune diseases include rheumatoid arthritis, scleroderma, polymyositis/dermatomyositis, lupus and sarcoidosis.

ILD caused by breathing in harmful substances: Some people are sensitive to develop ILD when they breath particles such as coal dust, silica, asbestos, tobacco smoke, chemicals, farm dust, birds specially pigeon and parrot. Silicosis, asbestosis and hypersensitivity pneumonitis are examples of this type of ILD.

Genetic ILD: A genetic ILD occurs when the disease is passed down among family members. These conditions include neurofibromatosis (a disease in which tumors grow on nerves) and Gaucher disease (marked by enlargement of internal organs, including the spleen and liver, and lesions on the bones).

Idiopathic ILD: Idiopathic means the cause is not known. Idiopathic ILD usually affects people over 60 years old. Idiopathic pulmonary fibrosis (IPF) is common example.

Interstitial lung disease ILD
How ILD is diagnosed?

A  doctor clinically examines a patient and suspects ILD and its causal factors. In order to confirm the diagnosis following tests may be required.

Lung function test will be done to check how well patient’s lungs are working

Imaging tests such as X-ray and a high-resolution computed tomography (HRCT scan)

Bronchoscopy: in this test bronchoscope is inserted through nose or mouth into lungs to look inside airways. In some cases biopsy of lungs is done in which doctor will take sample of lung tissue and sent it for biopsy.

How ILD is treated?

Treatments can not reverse the lung damage, but it can slow the disease progression which can  help you breathe more easily. Treatment for ILD is designed to preserve the ability of lungs to function and keep the disease from getting worse.

Doctors prescribe a few different types of treatments to manage interstitial lung disease:

Anti-inflammatory drugs, such as the steroid prednisone, can reduce swelling in the lungs. Immune-suppressing drugs, such as azathioprine, cyclophosphamide, and mycophenolate mofetil, may help stop the immune system attacks that damage the lungs. Antifibrotic drugs such as pirfenidone and nintedanib  may prevent further scarring in the lungs.

Supplemental oxygen is currently recommended in the international guidelines for treatment.

Pulmonary rehabilitation may help improve your activity levels and ability to exercise.

Lung transplant: Some people with severe cases of ILD have lung transplants to help prolong their lives.

Can interstitial lung disease be prevented?

There is no way to prevent idiopathic or genetic ILD, but it is possible to prevent some of the types with known causes. One can reduce your risk by:

  • Wearing a respirator (a mask that filters particles from the air) around harmful substances, such as asbestos, metal dusts or chemicals
  • Quitting smoking
  • Receiving immunizations for flu and pneumonia to help protect your lungs
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