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Interstitial Lung Disease

What is an Interstitial lung disease?

From the word itself, it is an inflammation of the lungs, involving primarily the interstitium or the tissue and space around the air sac of the lungs. The interstitium supports a wide array of capillaries and blood vessels, facilitating the free flow of blood carrying oxygen. Once there are problems in the interstitial tissues, it would result to diminished lung expansion and decreased supply of oxygen.


Intertitial lung disease

Picture showing alveoli of lung where Interstitial lung disease starts

Image source – medicine.ufl.edu

Interstitial Lung Disease Classification

Interstitial lung disease is a broad category of lung disorders. The following are some of the common disorders under interstitial lung disease:

  1. Idiopathic pulmonary fibrosis
  2. Connective tissue or autoimmune-related pulmonary fibrosis
  3. Hypersensitivity pneumonitis
  4. Sarcoidosis
  5. Eosinophilic granuloma
  6. Chronic eosinophilic granuloma
  7. Pulmonary vasculitis
  8. Bronchiolitis obliterans
  9. Lymphangioleiomyomatosis
  10. Familial interstitial pneumonia

Interstitial Lung Disease Symptoms

The two most common symptoms of interstitial lung disease are shortness of breath and dry cough because in the presence of an inflammation, there is decreased lung function. If the condition has been long-standing, it will eventually lead to scarring and thickening of the interstitial tissues of the lungs.

The thickened or hardened lung tissues are not able to expand as what normal tissues do; therefore, overall lung capacities are diminished. That is why, symptoms of this condition are related to reduced breathing ability which therefore leads to decreased distribution of oxygen throughout the body.

Causes

Interstitial lung disease is believed to have been caused by problems in the immune system wherein when an injury to lungs happen, it will trigger a healing response which is way beyond normal. When there is damage, to lung tissues, the body is capable of rebuilding the damaged structures by producing more cells to regenerate. However in the case of interstitial lung disease, more and more cells were produced thereby causing a thickening or hardening of the interstitium, making it difficult for oxygen and other gases to penetrate. Because of the decreased lung capacities, it all leads to decreased oxygen distribution throughout the body.

Occupational or Environmental Factors

Continued exposure to substances that gives off considerable amount of pollutants and toxins will result to damage to the lungs. This includes silica dust, asbestos fibers, grain dust, droppings from birds and other animals.

Radiation Therapy

People who have subjected themselves to radiation therapy either because of a lung or breast cancer usually experience lung damage after months or even years since the onset of the first treatment. However, the severity of the damage will depend on the how vast the area is being exposed, how long have it been exposed, what drugs have been used during the therapy, and whether or not there was an underlying lung disease.

Drug-Induced

Although drugs are designed to treat a certain disease, these drugs can also pose potential harm to vulnerable individuals. Medications used for chemotherapy (mothotrexate and cyclosphorine), heart conditions (amiodarone and propanolol), and some antibiotics (nitrofurantoin, sulfasalazine) are known to have the capacity of causing damage to the lungs.

Other Diseases

Certain systemic conditions can also lead to interstitial lung disease such as rheumatoid arthritis, systemic lupus, erythematosus, scleroderma, etc.

Interstitial Lung Disease Diagnosis

How is interstitial lung disease diagnosed is through a multiple-step process. It needs a thorough study of the individual’s family history, examination of the signs and symptoms, list all the current and previous medications, review of recent and past occupational exposure to environmental pollutants and toxins. After all the necessary information has been gathered, the following procedures will help in establishing the diagnosis:

  1. Physical examination – changes in the skin, joint, and fingernails can help in the evaluation. Assessment of lung sounds can also help in determining the problem.
  2. High resolution CT scan – results would usually be abnormal so this would help in establishing the diagnosis
  3. Pulmonary function test – with interstitial lung disease, pulmonary function test usually shows abnormal results
  4. Arterial blood gas (ABG) – the usual result of ABG analysis is sometimes normal or decreased
  5. Exercise Testing – this test may show that there is decreased level of oxygen during exercise
  6. Bronchoscopy – this is done to check for any inflammatory cells in the lungs. This process involves inserting a tube through the nostrils into the trachea to have a view of the airways.
  7. Lung biopsy – collection of sample tissues of the lungs for laboratory testing

Treatment

The goals of treatment for interstitial lung disease are to identify the specific disease in order to start the specific treatment right away because it is believed that current therapies are not really very effective in reversing the scarring of the lungs. That is why it is very important to detect the condition at an early onset. Another goals of therapy are to remove the problem source as much as possible to avoid recurrence, minimize the potential complications, and improve the patient’s condition and prevent deterioration of the quality of life.

Medications

These drugs aim to address breathing problems, reduce inflammation and suppress hyperactive immune systems. Medications for interstitial lung disease include corticosteroids, immunosuppressive drugs.

Oxygen Therapy

Deceased lung functions would lead to decreased lung expansion. This is the usual scenario with interstitial lung disease. In this case, the decreased lung functions would lead to decreased level of oxygen delivered to the lungs. Therefore supplemental oxygen therapy would be needed to maintain the sustainable level of oxygen needed for the body.

Pulmonary Rehabilitation

This program includes patient education, general conditioning, breathing exercises, energy conserving techniques, counseling, etc. This is often provided to ensure that patients reach their highest level of function.

Lung Transplant

Organ transplant is effective in end-stage interstitial lung disease. If the other methods of treatment are not effective and if the condition is severe, then lung transplant may be the most practical method to use.

Interstitial Lung Disease Prognosis

The prognosis for each patient also depends on the specific disease that has developed. In some cases, the disease would just stop progressing and show improvement of symptoms, especially if the causes have been identified early in the development of the disease. Other times, similar conditions would suddenly develop and develop either slowly or quickly into the end stage of the disease, which is quite difficult to manage.

Life Expectancy for Interstitial Lung Disease

The average life expectancy for individuals with interstitial lung disease is about two to five years. The fact that the lung tissues have undergone scarring or thickening, this is an irreversible condition. Therefore the diseases that develop secondary to this are quite irreversible. It is extremely important that early detection of the disease be made to establish the diagnosis as early as possible. It would follow then that early detection might somehow prolong life expectancy.

Living a life with lung disease can be very challenging both emotionally and physically. Major adjustments should be made in order at least make the afflicted individual live in its highest level of function. It is important for the patient to share his feelings and have someone to talk to. Joining a support group would be very helpful because one would be surrounded with people who somehow have had the same condition. Group members would share coping strategies, get new information with regard to new treatment methods, or just simply to have someone else listen to you as you pour out your feelings about anything in life

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