Interstitial Lung Disease – Prognosis, Life Expectancy, Symptoms


Interstitial lung disease or Diffuse Parenchymal lung disease is a large group of disorders that affects the interstitium (the tissue that surrounds and separates the air sac). It causes inflammation and progressive lung tissue scarring or Pulmonary Fibrosis.

Other parts of the lungs may also be affected, including: alveoli, trachea, bronchi, bronchioles, blood vessels and pleura. This kind of damage in the lungs is generally permanent and irreversible. As a result, it affects a person’s ability to breathe and lowers the amount of oxygen in the bloodstream.

ILD is a rare disease, but nowadays it has become more common. In the United States, a study reported that 80.9 per 100.000 men and 67.2 per 100,000 women suffer from interstitial lung disease. It is most common among adults between the ages of 40 and 70, but it also occurs in children (Childhood Interstitial Disease).

Studies have found that ILD had spread throughout the world, in many different racial and ethnic groups. People with autoimmune disease like lupus, rheumatoid arthritis and scleroderma are more often seen to have interstitial lung disease.

Types

Interstitial Pneumonia – caused by bacteria, fungi or viruses that infect the interstitium. The most common agent is mycoplasma pneumonia.

  1. Idiopathic Pulmonary Fibrosis- is severe, progressive scarring of the interstitium. The cause is unknown.
  2. Nonspecific interstitial pneumonitis- common to patients with autoimmune disease.
  3. Cryptogenic organizing pneumonia (COP)- ILD with pneumonia-like symptoms, but without presence of infection.
  4. Hypersensitivity pneumonitis- caused by exposure to different type of irritants, dust or molds.
  5. Acute interstitial pneumonitis- chronic but sudden type of ILD.
  6. Desquamative interstitial pneumonitis- most common among smokers and those with a history of smoking.
  7. Sarcoidosis- inflammation affects organs of the body. Usually the lymph nodes and the lungs. Granuloma (abnormal masses) alters the function of the affected organs.
  8. Asbetosis- caused by inhalation of asbestos.

Causes

Interstitial Lung Disease is caused by number of factors, including:

Environmental toxins and pollutants

Long term exposure to substances such as asbestos, silica dust, grain dust, gases, fumes, tobacco smoke, bird and animal droppings cannot be easily eliminated from the body and causes direct injury to the lungs.

Radiation therapy

Cancer patients undergoing long-term radiation therapy show symptoms of lung damage some years after the start of treatment.

Autoimmune Diseases

Autoimmune disorders like Systemic lupus erythematosus, Rheumatoid arthritis, Sarcoidosis and Scleroderma.

Medication

Some medicines are known to damage the lungs. Some drugs used for chemotherapy, heart diseases and antibiotics can also cause it.

Genes

Rare, about 8% of people suffering ILD get it genetically.

Idiopathic or unknown cause

Most common.

Cigarette smoking

Has been also associated with the disease development.

Symptoms

Interstitial Lung Disease involves inflammation and scarring of interstitial tissue. Tissues become thick and as a result, lungs can no longer function well.

Interstitial Lung Disease- Acute

Primary Symptoms includes:

  1. Shortness of breathing, is a result of reduced breathing capacity
  2. Non productive cough
  3. Crackles (abnormal chest sound)
  4. Cyanosis- this is a result of decreased oxygen levels in the blood, and may result in a bluish discoloration of the skin.

Interstitial Lung Disease- Chronic

In most cases, these symptoms become chronic. Signs are:

  1. Clubbing of the fingers
  2. Enlargement of the heart.

In some cases these symptoms are also involved:

  1. Fever
  2. Weight loss
  3. Muscle pain
  4. Joint pain

Diagnosis

In most cases, a number of diagnostic tests are used, since medical history and physical examination are not enough to confirm the disease. They are tools used by doctors to rule out interstitial lung disease. The following tests are necessary:

Chest X-ray

 Most of the time a chest x-ray is used as the first diagnostic step, and also used to detect the progression of disease.

Computerized Tomography (CT)

Images are taken from different angles to visualize cross sectional images of the lungs, including their internal structure. A CT scan determines the severity of lung damage. It also distinguishes if fibrosis or active inflammation has already taken place.


Oximeters

It measures the oxygen level of the blood.

Electrocardiogram (ECG)

Is done to visualize the heart’s structure and to show heart function. It is also used to determine if the disease has  affected the heart.

Spirometry

Is to test pulmonary function. It measures the amount of air your lungs can hold, and the airflow in and out of the lungs.

Bronchoalveolar Lavage

This procedure is non-invasive. The doctor will introduce a solution into an area of your lung and create suction. The collected output contains cells from the air sacs. It identifies the causes of interstitial lung diseases (infection, auto immune).

Surgical biopsy

Is a procedure where lung tissue samples are removed and sent to laboratory to be examined by a pathologist. For accurate diagnosis, large amounts of lung tissue are collected.

Treatment

Treatment for Interstital Lung Disease depends on what causes the disease and the overall health status of the patient. The scarring of interstitial tissues (Pulmonary Fibrosis) is irreversible, and at this time no treatment has been proven effective to stop the progression of the disease. The intent of this treatment is to prolong life, temporarily improve symptoms and slows the progression of the disease.

Medications

Patients with Interstitial Lung Disease are treated immediately, however, potential side effects of medications are also observed. Take these medications under your doctor’s supervision.

  1. Corticosteroid, like Prednisone, to reduced inflammation.
  2. Immune suppressing drugs- sometimes a combination of these drugs are also given to suppress the immune system. Some medications are used to decrease fibrosis and prolong survival.
  3. Antibiotics- your physician may prescribe antibiotics for infection.

Oxygen Therapy

Aids the client to have an easier time breathing and to prevent complications from Hypoxemia (lower level of oxygen in blood).

Pulmonary Rehabilitation

Is a program with the purpose of improving daily function and well-being of patients with Interstitial Lung Disease. This therapy also teaches proper breathing techniques for lung efficiency and to relieve breathing problems. It also provides lessons on lung disease, and nutritional and psychological counseling.

Lung transplant

Usually with a single lung, a lung transplant is considered as a last option for a patient with severe Interstitial Lung Disease and has not recovered after medical treatment. It is possible for patients under the age of 65 years and for those without other significant medical conditions.

Prognosis

Interstitial lung disease has a high rate of morbidity and mortality due to lung injury and fibrosis. Patients usually live for less than 3 years after being diagnosed. Since it is irreversible and fibrosis causes permanent structural damage to the lungs, medications cannot actually treat the disease, but only slow down the symptoms. This disease is most often fatal.

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9 thoughts on “Interstitial Lung Disease – Prognosis, Life Expectancy, Symptoms

  • June 25, 2014 at 5:52 am
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    I had a daughter who was nine years old that took a half a tab of augmentin Iantibiotic) 250 mg. it honey-combed her lung burned it up. They gave her chemo and steroid poinsoned my girl. A person can take stem cells and recover, this was l5 years ago, because conventional doctors would not work with alternative doctors my girl died……….no person should have to die, but that is the way this system is ran. Ask me how I feel as a parent, all the money that is pumped into arthritis and cancer is a joke. There are no cures coming til God himself brings them, he has given us wonderful treatments only to have them never brought to us…….I hate to hear there are no reversals or there is no help, yes there is….Wake up!!!

    Reply
    • November 8, 2014 at 7:48 pm
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      Tell me what to wake up too?

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    • June 26, 2015 at 3:28 am
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      so sad & heartbreaking for a family to endure.

      Reply
  • March 8, 2015 at 9:14 pm
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    I hate to say it, they will never find a cure for cancer (or any other disease’s). Because they would not recieve all that money. Do you think they would forfit over a billon dollars a year. Thats bonuses and vacations and what not. Sorry to hear about you daughter. God bless her.

    Reply
  • September 24, 2015 at 12:11 am
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    My 55-year-old son was diagnosed with ILD in the past year. I am very scared. If someone has something positive to say, please tell me. I am so hopeful of learning that it is curable. I have lost a husband and my oldest son (not with this) in the past year, and the thought of losing another son breaks my heart. A child is not supposed to die before a parent.

    Reply
    • December 4, 2015 at 4:39 am
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      Don’t lose hope, but be glad for the time you’ve had. I’ll agree that there’s nothing quite like the loss of a child, but please relish in the fact yours made it 50+ years. :)
      (I wasn’t fortunate enough to see 2 children make it passed their first year of life)
      Again, do not lose hope!!

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    • February 9, 2016 at 3:28 am
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      I just got a diagnosis of C.O.P. , one of the interstitial lung diseases. They will treat it with prednisone for months or up to who knows how long. And the illness might resolve itself from that treatment. Yesterday I thought I would eventually die of it. Today, new lease on life. The key, I think, is finding a treatment center that specializes in interstitial lung diseases. Precise diagnosis is crucial. My pulmonologist presented my case to the San Francisco University Hospital interstitial lung program. The clinic docs spent two and a half hours reviewing all of my test results and agreed that Cryptogenic (unknown cause) Organizing Pneumonia is my problem. Only about 7% of diseased patients die of this, and usually the elderly or folks with serious health problems. I’m 78, but healthy enough to beat this. I know it is scary. There are new findings almost every day. Bless your heart, and keep the faith. Keep talking with others on the list serves. they have helped me a great deal. Best of luck. Nancy

      Reply
  • February 10, 2016 at 3:32 am
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    Im 37 and was diagnosed with COP with auto immune disorder 2 years ago. I’ve been through the treatmemts….not getting better. It has now progressed to my heart. Needless to say, I’m trying to deal with the fact that it will be a high possibility that I will not see 40. I have been struggling even more with having to share this news with my family and close friends. So, I keep all the negative regarding this disease between myself and my therapist. I have decided to just live the best life i can while i can. The rest is up to God!

    Reply
  • February 24, 2016 at 10:08 pm
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    My wife has ILD We have six children from 16 years to one and half four little boys and two teenage girls. My wife just had another MRI this morning and the technician looked very concerned. She has already had a large section of her right lung removed. A lot of trips to the hospital in the last two years.
    I s there better treatment places in the country. We are going to Christ Hospital In Cincinnati,Ohio. They seem very good but the doctors are,tn giving us much to hope for. Please help

    Reply

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