Are Interstitial Lung Disease And Pulmonary Fibrosis The Same Thing?

Are Interstitial Lung Disease And Pulmonary Fibrosis The Same Thing?

Interstitial lung disease and pulmonary fibrosis are general terms to describe inflammatory and fibrotic disorders that affect the lung tissue (interstitium). Pulmonary fibrosis is an interstitial lung disease in which chronic inflammation occurs, which is accompanied by an uncontrolled healing response that causes progressive scarring or thickening of tissues between the lungs alveoli and air sacs. In this the body’s own immune response is compromised that leads to fibrosis of the lung tissue. It is mostly used synonymously with interstitial lung disease. It is a type of interstitial lung disease.

The disease affects men twice more than women. The patients are usually in the age group of 40 to 70 years of age. The interstitial disease cannot be completely cured, but its progression can be delayed with the use of appropriate treatment. It is usually harder to treat in older patients. Both incidences of interstitial lung disease and mortality due to ILD increase as the age progresses. The disease affects the alveoli, airways (trachea, bronchi and bronchioles), interstitium, blood vessels, and pleura (outside lining of the lung). Depending on the type of interstitial lung disease, a person can develop varying degrees of fibrosis, inflammation and different symptoms. Sometimes the damage caused by ILD can be permanent and irreversible and does not respond to treatment but only worsens with time.

Causes And Symptoms of Interstitial Lung Disease

Interstitial lung disease is usually described as chronic, nonmalignant and noninfectious in nature. Although symptoms vary from person to person, but the basic terminology remains inflammation in the small airways of the lungs that will cause scarring in the lung tissue. Due to this scarring the lung tissue hardens and there is not enough expansion of the lungs while breathing, which leads to respiratory discomfort.

Some of the known causes that lead to interstitial lung disease are radiation exposure, connective tissue diseases such as scleroderma, sarcoidosis, genetic conditions and various pharmaceutical medications. Exposure to toxic agents such as asbestos, silica, metal, wood dusts and antigens can lead to interstitial lung disease.

The symptoms of interstitial lung disease are caused by abnormalities that characterize the disease into respiratory symptoms, symptoms caused by chest abnormalities, symptoms caused by changes in pulmonary function and those caused by microscopic patterns of inflammation and fibrosis. These symptoms include chest pains, difficulty in breathing or shortness of breath, dry chronic cough, lethargy, weakness, fatigue, tendency to tire easily even with moderate exercise, pain in muscle and joints, weight loss, clubbing of fingers and toes, edema in the lower legs, fluid buildup in the lungs (pulmonary edema), sleep apnea (difficulty in sleeping), headaches, fever and allergic reactions in some cases.

An advanced stage of interstitial disease will present with breathlessness, ongoing fatigue, rapid breathing and confusion due to increased levels of carbon dioxide in the blood.

Diagnosis And Treatment of Interstitial Lung Disease

The diagnosis is made on the basis of medical history and tests such as high resolution computerized tomography (HRCT) scan that provides a detailed cross section of the chest. Pulmonary function tests are performed to determine the functioning of the lung. Exercise tests, bronchoscopy, bronchovascular lavage, chest X ray, blood gases test, help in confirming the diagnosis.

The treatment plan includes the use of antifibrotics that help reduce the development of scar tissue. It helps in reducing the progression of lung damage. Colchicines and penicillamine have proven to be beneficial in clinical trials. In some cases pirfenidone has also shown good results in phase II trial. Corticosteroids reduce inflammation of lungs; however the benefits are usually temporary. Also corticosteroids taken in the long run can cause certain side effects such as glaucoma; bone loss, high blood sugar, and poor wound healing and increased susceptibility to infections. Cytotoxic drugs are also used to treat interstitial lung disease when steroid treatment fails. A lung transplant is necessary in patients where all other methods of treatment have failed. Oxygen therapy and pulmonary rehabilitation also help patient recover emotionally as well as medically.

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Pramod Kerkar, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:October 20, 2018

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