Description

Interstitial (in-tur-STISH-ul) lung disease, also called interstitial lung disease describes a large group of conditions. Most of these conditions cause inflammation and progressive scarring of the lung tissue. As part of this process, the lung tissue thickens and hardens, which makes it difficult for the lungs to expand and fill with air.

At some point, the scars of the interstitial lung disease makes it more difficult to breathe and get enough oxygen into the bloodstream. Many people with ILD shortness of breath with activity and you may have an annoying dry cough.

Interstitial lung disease can have many causes, including long-term exposure to hazardous materials such as asbestos. Some types of autoimmune diseases, such as rheumatoid arthritis, also can cause interstitial lung disease. But the cause is not known, at times. ILD can have many causes, so the treatment varies.

The disease can get worse slowly or rapidly, at a pace that often can't be predicted. Once lung scarring occurs, it usually is not reversible. The treatment focuses on keeping more of the healing of the occurrence, the management of the symptoms and the quality of the life better. The drugs can decrease the damage of interstitial lung disease, but many people never fully utilize their lungs again. Lung transplantation is an option for some people who have interstitial lung disease.

Symptoms

The main symptoms of interstitial lung disease are:

  • Shortness of breath at rest or difficulty breathing that is worse with physical activity.
  • Dry cough.

When to see a doctor

By the time they show symptoms of certain types of interstitial lung disease, with a duration of lung damage has already occurred. That is why it is important that you consult your health care professional at the first sign of respiratory problems. Many other conditions of the ILD may affect the lungs. Arrive early and correct diagnosis is important for proper treatment.

Causes

Interstitial lung disease seems to occur when an injury to the lungs triggers a healing response that is not correct. Typically, your body creates just the right amount of tissue to repair damage. But in ILD, the repair process does not work correctly. Tissue in and around the lungs, air sacs, called alveoli, are inflamed, scarred and thickened. This makes it more difficult for oxygen to pass into your bloodstream.

There are many types of interstitial lung disease. Are generally grouped by known or unknown causes:

  • Their work or the work environment.
  • Underlying systemic condition.
  • Certain types of medications, or radiation.
  • There is No known cause.

Some interstitial lung diseases may be related to smoking.

Their work or the work environment

Long-term exposure to some toxins and pollutants that can harm the lungs. For example:

  • Pneumoconiosis. Pneumoconiosis (noo-moe-koh-nee-O-sis) refers to a type of interstitial lung disease caused by the inhalation of certain types of powder of work or in another environment for a long time. Diseases in this group can cause lung scarring and injuries over time, which leads to shortness of breath and poor capacity to take in oxygen. These symptoms may not be reversible. The disease is often named after the type of exhibition or work role itself. They include diseases such as the coal miner's lung, caused by the inhalation of coal dust and asbestosis, caused by the inhalation of asbestos particles. These diseases also include silicosis, caused by the inhalation of silica dust.
  • Hypersensitivity pneumonitis. This inflammation of the lungs caused by the inhalation of airborne irritants, often with the proteins of living things. The most common of these airborne irritants are birds of the protein, mold, or bacteria. Conditions of this sort are also often named after the type or source of exposure. For example, the pigeon-breeder's or bird lovers of the disease involves being exposed to birds of the protein, and the farmer's lung involves being exposed to moldy hay. People with this type of lung inflammation can be achieved better to stay away from the irritant. But this type of inflammation of the lungs can also get worse and cause more permanent damage if people are still breathing in irritant.

Of the underlying systemic condition

Certain diseases or conditions that can lead to interstitial lung disease. For example:

  • Diseases of the connective tissue. These include autoimmune diseases, such as rheumatoid arthritis, scleroderma, and mixed connective tissue disease. These diseases result from an immune response that is not correct and can cause inflammation of the tissues and the formation of scars on the body, including the lungs.
  • Sarcoidosis. This is a disease that includes the growth of tiny collections of inflammatory cells called granulomas in any part of your body, most commonly the lungs and lymph nodes. Other commonly affected organs are the eyes, the skin, the heart, the spleen and the liver.

Medications, radiation,

Certain types of medications that can cause interstitial lung disease in some people. This may or may not be reversible, based on the type and duration of the exposure.

The drugs most commonly associated with ILD are:

  • The chemotherapy drugs. The drugs designed to kill cancer cells, such as bleomycin, gemcitabine, and the point of control immune inhibitors, can damage the lung tissue.
  • Drugs for the heart. Some medicines used to treat irregular heartbeats such as amiodarone (Nexterone, Pacerone), can damage the lung tissue.
  • Some antibiotics. Nitrofurantoin (Macrobid, Macrodantin, and others), and daptomycin may cause lung damage.
  • Anti-inflammatory medications. Certain anti-inflammatory medications, such as methotrexate (Trexall, Xatmep, others) or sulfasalazine (Azulfidine), can damage the lungs.

Targeted radiation to the chest during the treatment for certain types of breast cancers, and cancers of the lung, for example — can lead to injuries or long-term scarring in some people. How severe the damage is, you can depend on:

  • The amount of lung that was exposed to radiation.
  • The total amount of radiation given.
  • If chemotherapy is also used.
  • If there is underlying lung disease.

There is No known cause

The list of substances and conditions that can lead to interstitial lung disease is long. Even so, in some people, the cause is never found. Conditions without a known cause are grouped under the label of the idiopathic interstitial pneumonias. For example:

  • The idiopathic pulmonary fibrosis, also called the IPF. The FPI is a typical progressive lung disease that occurs when lung tissue becomes damaged and scarred — what is known as fibrosis. Idiopathic means that the cause is not known. The FPI can be seen in the image and biopsy if a lung biopsy is taken. This thickened, stiff tissue makes it more difficult for the lungs to function properly. The most common type of ILD, IPF is often worse, and cannot be reversed.
  • Organizing pneumonia cryptogenic, also called the police. The COP is a rare lung condition in which the small airways, called bronchioles, and the little exchange of air sacs, called alveoli, become inflamed. This inflammation makes it difficult to breathe. Imaging tests show the presence of pneumonia, but the COP is not an infection, and the cause is not known. Scarring or fibrosis is rare, but can occur in some patients if the condition becomes.
  • Nonspecific interstitial pneumonia. This type of interstitial lung disease causes cells to inflamed or scar tissue to build up in the spaces between the air sacs in the lungs. It is more likely to occur in people with connective tissue diseases, but may also be linked to other conditions.

Risk factors

The factors that may make you more susceptible to contracting the disease interstitial lung include:

  • Age. ILD is much more likely to affect adults, although infants and children sometimes have the disease.
  • The exposure to toxins in the workplace or in the environment. Working in the mining, agriculture, or construction, or for any reason are not exposed to contaminants can damage the lungs, increases the risk of developing interstitial lung disease.
  • The habit of smoking. Some forms of ILD are more likely to occur in individuals with a history of smoking. The habit of smoking can make the disease worse, especially if you also have emphysema.
  • The radiation and chemotherapy. To have radiation treatments to the chest or the use of certain chemotherapy drugs increases the risk of lung disease.
  • A connective tissue disease. This includes autoimmune diseases that may increase your risk of ILD.

Complications

Interstitial lung disease can lead to a series of life-threatening complications, including:

  • High blood pressure in the lungs, known as pulmonary hypertension. Unlike the systemic arterial hypertension, this condition only affects the arteries of the lungs. Scar tissue, or low oxygen levels restrict the smallest blood vessels, limiting blood flow in your lungs. This increases the pressure within the pulmonary arteries and may worsen the exchange of oxygen, the reduction of oxygen levels in the blood. Pulmonary hypertension is a serious disease that can worsen over time, causing the right side of his heart to fail.
  • Right-sided heart failure, also known as cor pulmonale. This serious condition occurs when the heart's lower right chamber, also known as the right ventricle must pump harder than usual to the blood flow through a blocked pulmonary artery. Finally, the right ventricle fails of the extra stress. This is often due to pulmonary hypertension.
  • The respiratory failure. In the final phase of the chronic, interstitial lung disease, respiratory failure occurs when the very low levels of oxygen in the blood, along with the increase of the pressure in the pulmonary arteries and the right ventricle, causes the heart to fail.

Prevention

To prevent interstitial lung disease, avoid exposure to toxins in the workplace, such as asbestos, coal dust and silica dust. Also, avoid exposure to toxins in the environment, such as the bird of the protein, mold, mildew and bacteria. If you must be around these toxins, protect yourself with the use of a respirator. Other ways of preventing the ILD include not smoking and avoid second-hand smoke.

If you have an autoimmune disease or are taking medicines that increase your risk of getting the ILD, talk with your health care professional about the steps you can take to prevent the ILD. Also, to get vaccinated due to respiratory infections can make the symptoms of ILD worse. Make sure you receive the vaccine against pneumonia and the flu each year. Also, ask your health care provider about the vaccine whooping cough, COVID-19, and respiratory syncytial virus, also called RSV.

Diagnosis

Find the cause of interstitial lung disease can be difficult, and sometimes the cause can't be found. Many conditions fall into the category of ILD. In addition, the symptoms of a wide range of medical conditions that may appear to be of ILD. Health care professionals should rule out these conditions before making a diagnosis.

Some of the following tests may be necessary.

Laboratory tests

  • Blood tests. Certain blood tests may detect proteins, antibodies and other markers of autoimmune diseases or inflammatory responses to environmental exposures, such as those caused by molds or birds of the protein.

Imaging tests

  • The computed tomography scan, also called a CT or cat scan. This test image is key to the diagnosis of interstitial lung disease. Sometimes the first test in the diagnosis. CT scanners produce 3D images of the internal structures. A high-resolution CT scan can be particularly useful to find out how much lung damage that there is. You can display details of fibrosis, which can help to reduce the diagnosis and guide treatment decisions.
  • The echocardiogram. An echocardiogram uses sound waves to visualize the heart. You can create pictures of your heart structures, and videos that show how well your heart is working. This test measures the amount of pressure in the right side of his heart.

Pulmonary function tests

  • Spirometry and diffusion capacity. This quiz requires you to breathe quickly and with force through a tube connected to a machine. The machine measures the amount of air that the lungs can hold and how quickly you can move air out of your lungs. It also measures the ease of oxygen can pass from the lungs to the blood stream.
  • Oximetry. A small device that's placed in one of your fingers to measure the level of oxygen in the blood. This test may be done at rest or with activity to monitor the course of the lung disease and find out how serious it is.

The lung tissue analysis

Sometimes the interstitial lung diseases can be diagnosed only by a small amount of lung tissue in a laboratory of what is known as a biopsy.

Your doctor or other health care provider may take a sample of tissue using one of these methods:

  • The bronchoscopy. In this procedure, the healthcare provider removes small samples of tissue (usually not larger than the head of a pin. This is done through the use of a small flexible tube, also known as a bronchoscope, which is passed through the mouth or nose into the lungs. The risks of bronchoscopy are usually of minor importance, namely, a sore throat for a limited time and the hoarseness of the bronchoscope. But the tissue samples are sometimes too small to make a diagnosis.
  • The bronchoalveolar lavage fluid. In this procedure, the doctor injects a tablespoon of sterile salt water through a bronchoscope in a section of his lung, and then suctions it out immediately. The solution that has been removed contains cells of your air sacs. Although bronchoalveolar lavage samples a larger area of the lungs that other procedures to do so, you may not give enough information to figure out what the cause of the pulmonary fibrosis.
  • Surgical biopsy. Although this is a more invasive procedure with potential complications, it is often the only way to get a large enough tissue sample to make the correct diagnosis. General anesthesia is used for this test. Surgical instruments and a tiny camera is inserted through one or more small incisions between the ribs. The camera allows the surgeon to see the lungs on a video monitor, while the removal of tissue samples from the lungs.

Treatment

Scarring of the lung that has already occurred in the interstitial lung disease cannot be reversed, and the treatment does not always prevent the disease from getting worse. Some treatments may make symptoms better for a short period of time, or delay the disease. Other help to maintain the quality of life.

Because many of the different types of scars of the diseases have no approved or proven therapies, clinical trials may be an option to get an experimental treatment.

Drugs

An intense research to find the treatment options for specific types of interstitial lung disease is in progress. The treatment can vary depending on the cause of ILD and the damage that has happened in the lungs. Using the latest scientific evidence, your health care professional may recommend:

  • Corticosteroid medicines. In the first place, many people diagnosed with interstitial lung disease idiopathic treated with corticosteroids, namely, prednisone (Prednisone Intensol, Rays). Sometimes people are treated with other medications that suppress the immune system. Depending on the cause of ILD, these drugs can delay or even prevent the disease from getting worse.
  • Drugs that slow the deterioration of idiopathic pulmonary fibrosis. Pirfenidone (Esbriet) and nintedanib (Ofev) are medications that can decrease the rate at which the IPF is compounded. Ofev has also been approved for people with pulmonary fibrosis, which is getting worse due to other types of interstitial lung disease. Side effects of both drugs are common. Talk with your health care professional about the pros and cons of these medications.
  • Medicines that reduce stomach acid. Gastroesophageal reflux disease, also known as GERD, affects the majority of people with idiopathic pulmonary fibrosis. GERD is linked to worse lung damage. If you have symptoms of acid reflux, your doctor may prescribe ways to treat GERD reduce stomach acid.

Oxygen therapy

The use of oxygen cannot stop damage to the lungs, but it can:

  • Facilitate breathing and exercise.
  • To prevent or reduce the complications of low levels of oxygen in the blood.
  • Lowering the blood pressure in the right side of his heart.
  • Make your dream and the sense of well-being better.

You are more likely to get oxygen when you sleep or exercise, although some people can use it throughout the day.

Pulmonary rehabilitation

The goal of pulmonary rehabilitation is to make you more able to function and live a full and satisfying life. That is why the pulmonary rehabilitation programs focus on:

  • Learn more about their lung disease.
  • Exercise, so that you can become more physically active for long periods of time.
  • The breathing techniques which make your lungs more efficient.
  • Emotional support.
  • Nutritional advice.

Surgery

A lung transplant may be an option of last resort for some people with severe interstitial lung disease when other treatment options have not helped.

Lifestyle and home remedies

You must actively participate in their own treatment and staying as healthy as possible when you're living with interstitial lung disease. For that reason, it is important to:

  • Learn about your disease. Understanding your condition and how it can be treated can help you to decide about your care. Including family members and friends can help you learn to your needs.
  • Stop smoking. If you have lung disease, the best thing you can do for yourself is to quit smoking. Talk with your health care professional about the options for smoking cessation, including programs to help you stop smoking. These programs use a variety of techniques proven to help people quit smoking. And due to that the second-hand smoke can also cause damage to the lungs, do not allow people to smoke around you.
  • Avoid exposure in the work or for hobbies. When possible, stay away from substances that can irritate the lungs. Ask your health professional for more information and advice.
  • Eat well. If you have lung disease, you can lose weight, because it is not comfortable to eat, and because of the extra energy you need to breathe. Try to eat a nutritionally rich diet that contains enough calories. A dietitian can give you more guidelines for a healthy diet.
  • Get vaccinated. Respiratory infections can make the symptoms of ILD worse. Be sure to get the vaccine against pneumonia and the flu each year.

Coping and support

Living with a chronic lung disease is emotionally and physically demanding. You may need to change their daily routines and activities — sometimes a lot — such as breathing problems get worse, or health care needs become more important. Feelings of fear, anger and sadness are so typical that mourn the loss of their old way of life and the concern about what's next for you and your family.

Share your feelings with your loved ones and your health care professional. Talking openly can help you and your loved ones deal with the emotional challenges of their disease. Also, clear communication can help you and your family plan for your needs if your illness gets worse.

Think about joining a support group where you can talk with people who are facing challenges like yours. The members of the group can share coping strategies, the exchange of information about new treatments or just listen as they express their feelings. If a group is not for you, you may want to talk with a counselor on a one-to-one fit.

Preparing for your appointment

Probably first see your family health care provider about your symptoms. Your family health care provider may refer you to a pulmonologist, a doctor who specializes in lung diseases.

What you can do

Before your appointment, you might want to prepare the answers to these questions:

  • What are the symptoms and when do they start?
  • You are receiving treatment for other medical conditions?
  • What medications and supplements that have been adopted in the past five years, including medicines obtained without a prescription and street drugs?
  • What are all the occupations that I have had, even if only for a couple of months?
  • Does any members of your family have a duration of lung disease of any kind?
  • Have you ever had chemotherapy or radiation treatments for cancer?

Let your pulmonologist to know if you have had any chest x-rays, ct scan, or other tests for the health care professional can apply the results before your visit. The images are more important that the report only. The pulmonologist can make a diagnosis by comparing a former test image with those of a current test.

What to expect from your doctor

Your doctor or other health care professional can ask some of these questions:

  • Do your symptoms occur all the time, or do they seem to disappear and then come back?
  • Have you recently had contact with air conditioners, humidifiers, pools, hot tubs, or damaged by the water of the walls or the carpet?
  • Do you have a pet bird or feather-containing items such as pillows of down or feathers?
  • Are you exposed to mold or construction dust in your home or in other places where you spend a lot of time?
  • Have any close relatives, friends, or coworkers have been diagnosed with a lung condition?
  • Do you have any family history of lung disease?
  • Does your work history include regularly work with or be close to toxins and pollutants, such as asbestos, silica powder or grain dust?
  • Do you or do you smoke? If so, how much and for how long? If not, you spent a lot of time around people who smoke?
  • Have you been diagnosed or treated for other medical conditions, especially arthritis, or rheumatic diseases?
  • Do you have symptoms of gastroesophageal reflux disease, also known as GERD, such as heartburn?
Symptoms and treatment of Interstitial lung disease