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Lung Disease

Chronic Lung Diseases: Causes and Risk Factors

When you think of chronic lung disease, you may think of lung cancer, but there are actually many different types. Altogether, lung diseases accounted for more than 1 million deaths in the U.S. in 2010, according to the National Heart, Lung, and Blood Institute (NHLBI).
These types of lung diseases may affect your airways, lung tissues, or circulation of blood in and out of your lungs. Here are the most common types, their causes and risk factors, and potential symptoms that may signal the need for medical attention.
Asthma
Asthma is one of the most common types of chronic lung disease. When triggered, your lungs become swollen and narrow, making it harder to breathe. Symptoms include:

wheezing

being unable to take in enough air

coughing

feeling tightness in your chest

If you experience these symptoms, it’s important to see a doctor right away. Triggers may include allergens, dust, pollution, stress, and exercise.
Asthma usually starts in childhood, though it can begin later. It can’t be cured, but medications can help control symptoms. The disease affects about 26 million Americans and tends to run in families.
Most people with asthma can manage it fine and enjoy full and healthy lives. Without treatment, though, the disease can be deadly. It kills about 3,300 people annually in the United States.
Doctors don't know yet why some people get asthma and others don’t. But they believe that genetics play a large role. If someone in your family has it, your risk goes up. Other risk factors include:

having allergies

being overweight

smoking

being exposed frequently to pollutants

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Chronic obstructive pulmonary disease

Chronic obstructive pulmonary disease (COPD) is a chronic lung disease in which your lungs become inflamed, making breathing more difficult. The inflammation leads to an overproduction of mucus and a thickening of the lining of your lungs. The air sacs, or alveoli, become less efficient at bringing oxygen in and sending carbon dioxide out.

People with COPD typically have one or both of the following conditions:

Emphysema: This disease damages the air sacs in your lungs. When healthy, the air sacs are strong and flexible. Emphysema weakens them and eventually causes some to rupture.

Chronic bronchitis: You may have experienced bronchitis when you had a cold or sinus infection. Chronic bronchitis is more serious, as it never goes away. It causes inflammation of the bronchial tubes in your lungs. This increases mucus production.

Symptoms of emphysema include:

shortness of breath

wheezing

the feeling of not being able to get enough air

Symptoms of chronic bronchitis include:

frequent coughing

coughing up mucus

shortness of breath

chest tightness

COPD is an incurable, progressive disease most often caused by smoking, though it also has a powerful genetic component. Other risk factors include:

exposure to secondhand smoke

air pollution

occupational exposure to dust, fumes, and smoke

Symptoms of COPD get worse over time. However, treatments can help slow progression.

Interstitial lung disease

A number of different lung diseases fit under the umbrella term "interstitial lung disease." Interstitial lung diseases include over 200 types of lung disorders. A few examples:

sarcoidosis

idiopathic pulmonary fibrosis (IPF)

Langerhans cell histiocytosis

bronchiolitis obliterans

The same thing happens with all of these diseases: The tissue in your lungs becomes scarred, inflamed, and stiff. Scar tissue develops in the interstitium, which is the space in your lungs between the air sacs.

As the scarring spreads, it makes your lungs more rigid, so they’re unable to expand and contract as easily as they once did. Symptoms include:

a dry cough

shortness of breath

difficulty breathing

You may be more at risk if someone in your family had one of these diseases, if you smoke, and if you’re exposed to asbestos or other inflammatory pollutants. Some autoimmune diseases have also been linked to interstitial lung disease, including rheumatoid arthritis, lupus, and Sjogren’s syndrome.

Other risk factors include going through radiation for cancer treatments, and taking some medications like antibiotics and prescription heart pills.

These diseases are incurable, but newer treatments hold promise for slowing their progression.

Pulmonary hypertension

Pulmonary hypertension is simply high blood pressure in your lungs. Unlike regular high blood pressure, which affects all the blood vessels in your body, pulmonary hypertension affects only those blood vessels between your heart and lungs.

These blood vessels become narrowed and sometimes blocked, as well as stiff and thick. Your heart has to work harder and push the blood more forcefully, which increases the blood pressure in lung arteries and capillaries.

Gene mutations, drugs, and congenital heart diseases can all cause pulmonary hypertension. Other lung diseases like interstitial lung disease and COPD may also be to blame. If left untreated, the condition can lead to complications like blood clots, arrhythmia, and heart failure.

Risk factors for pulmonary hypertension include:

being overweight

having a family history of the disease

having another lung disease

using illegal drugs

taking certain medications, like appetite-suppressant drugs

Symptoms include:

shortness of breath

chest pain

dizziness

fatigue

rapid heart rate

edema (swelling) in your ankles

This disease can't be cured, but treatments can help lower the pressure to a more normal level. Options include medications like blood thinners, diuretics, and blood vessel dilators. Surgery and transplantation are reserved as last resorts.

Cystic fibrosis

Cystic fibrosis is an inherited lung disease that affects newborn children. It changes the makeup of mucus in the body. Instead of being slippery and watery, mucus in a person with cystic fibrosis is thick, sticky, and excessive.

This thick mucus can build up in your lungs and make it more difficult to breathe. With so much of it around, bacteria grow more easily, increasing risk of lung infections.

Symptoms usually start in infancy and include:

chronic coughing

wheezing

shortness of breath

coughing up mucus

recurring chest colds

extra salty sweat

frequent sinus infections

According to the NHLBI, it can affect other organs in addition to the lungs, including your liver, intestines, sinuses, pancreas, and sex organs.

Doctors know that cystic fibrosis is caused by a gene mutation that normally regulates the level of salt in cells. The mutation causes this gene to malfunction, changing the makeup of mucus and increasing salt in sweat. There is no cure for the disease, but treatment eases symptoms and slows progression.

Early treatment is best, which is why doctors now regularly screen for the disease. Medications and physical therapy help loosen mucus and prevent lung infections.

Chronic pneumonia

Pneumonia is a lung infection caused by bacteria, viruses, or fungi. Microorganisms grow and thrive in the lungs, creating difficult symptoms. The air sacs become inflamed and may fill up with fluid, which disrupts the flow of oxygen. Most of the time people recover within a few weeks. Sometimes, though, the disease hangs on, and may even become life-threatening.

Pneumonia can attack anyone, but it’s most likely to develop in people whose lungs are already vulnerable because of:

smoking

a weakened immune system

another illness

surgery

Many times, pneumonia can be cured. Antibiotics and antiviral medications can help, and with time, rest, and fluids, the disease will often go away. In some cases, though, it can come back again and again, becoming a chronic disease.

Symptoms of chronic pneumonia include:

coughing up blood

swollen lymph nodes

chills

lasting fever

The symptoms may continue for a month or longer. Even if you take antibiotics, the symptoms may return when you finish them.

If regular treatments don't work, your doctor may recommend hospitalization so you can have access to additional treatment and rest. Possible complications of chronic pneumonia include lung abscesses (pus pockets in or around your lungs), uncontrolled inflammation in your body, and respiratory failure.

Lung cancer

Lung cancer is a disease in which the cells in your lungs grow abnormally, gradually developing tumors. As the tumors get bigger and more numerous, they can make it more difficult for your lungs to do their job. Eventually, the cancerous cells can spread to other areas of your body.

Lung cancer is the leading cause of cancer death in the U.S., according to the Mayo Clinic. It can grow for awhile without creating any symptoms. When symptoms do develop, they are often thought to be caused by other conditions. A nagging cough, for example, can be a symptom of lung cancer, but it can be caused by other lung diseases as well.

Other possible symptoms of lung cancer include:

wheezing

shortness of breath

unexplained weight loss

coughing up blood

Those most at risk include those who:

smoke

are exposed to dangerous chemicals by inhalation

have a family history of lung cancer

have other types of cancer

Treatment depends on the type of lung cancer and its severity. Your doctor will typically create a plan that includes surgery to remove the cancerous part of the lung, chemotherapy, and radiation. Some medications can also help target and kill cancerous cells.

How to protect your lungs

To increase your odds of avoiding chronic lung disease, consider these tips:

Don't smoke, or quit smoking. Avoid secondhand smoke.

Try to reduce your exposure to pollutants in the environment, at work, and in your home.

Exercise regularly. Aerobic exercise that increases your heart rate is best.

Eat a nutritious diet.

Get regular checkups with your doctor.

Be sure to get a flu shot every year, and after you turn 65, get a pneumonia shot.

If you’re at risk for lung cancer, ask your doctor about screening options.

Test your home for radon gas.

Wash your hands regularly, avoid touching your face, and stay away from individuals who are sick.

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Symptoms

Chronic cough: A cough that you have had for a month or longer is considered chronic. This is an important early symptom that tells you something is wrong with your respiratory system.

Shortness of breath: It's not normal to experience shortness of breath that doesn't go away after exercising, or that you have after little or no exertion. Labored or difficult breathing—the feeling that it is hard to breathe in out—is also a warning sign.

Chronic mucus production: Mucus, also called sputum or phlegm, is produced by the airways as a defense against infections or irritants. If your mucus production has lasted a month or longer, this could indicate lung disease.

Wheezing: Noisy breathing or wheezing is a sign that something unusual is blocking your lungs' airways or making them too narrow.

Coughing up blood: If you are coughing up blood, it may be coming from your lungs or upper respiratory tract. Wherever it's coming from, it signals a health problem.

Chronic chest pain: Unexplained chest pain that lasts for a month or more—especially if it gets worse when you breathe in or cough—also is a warning sign.

Causes

What Causes Pulmonary Fibrosis?

In most cases, there is no known cause for the disease.

Things that may increase the risk of pulmonary fibrosis include:

Cigarette smoking

Certain viral infections

Exposure to environmental pollutants, including silica and hard metal dusts, bacteria and animal proteins, and gases and fumes

The use of certain medicines

Genetics. Some families have at least two members who have pulmonary fibrosis.

Gastroesophageal reflux disease (GERD). GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.

Diagnosis

Identifying and determining the cause of interstitial lung disease can be challenging. A large number of disorders fall into this broad category. In addition, the signs and symptoms of a wide range of medical conditions can mimic interstitial lung disease, and doctors must rule these out before making a definitive diagnosis.

Some of the following tests may be necessary.

Laboratory tests

Blood tests. Certain bloodwork can detect proteins, antibodies and other markers of autoimmune diseases or inflammatory responses to environmental exposures, such as those caused by molds or bird protein.

Imaging tests

Computerized tomography (CT) scan. This imaging test is key to, and sometimes the first step in, the diagnosis of interstitial lung disease. CT scanners use a computer to combine X-ray images taken from many different angles to produce cross-sectional images of internal structures. A high-resolution CT scan can be particularly helpful in determining the extent of lung damage caused by interstitial lung disease. It can show details of the fibrosis, which can be helpful in narrowing down the diagnosis and in guiding treatment decisions.

Echocardiogram. A sonogram for the heart, an echocardiogram uses sound waves to visualize the heart. It can produce still images of your heart's structures, as well as videos that show how your heart is functioning. This test can evaluate the amount of pressure occurring in the right side of your heart.

Pulmonary function tests

Spirometry and diffusion capacity. This test requires you to exhale quickly and forcefully through a tube connected to a machine that measures how much air your lungs can hold, and how quickly you can move air out of your lungs. It also measures how easily oxygen can move from the lungs into the bloodstream.

Oximetry. This simple test uses a small device placed on one of your fingers to measure the oxygen saturation in your blood. It may be done at rest or with activity to monitor the course and severity of lung disease.

Lung tissue analysis

Often, pulmonary fibrosis can be definitively diagnosed only by examining a small amount of lung tissue (biopsy) in a laboratory.

The tissue sample may be obtained in one of these ways:

Bronchoscopy. In this procedure, your doctor removes very small tissue samples — generally no larger than the head of a pin — using a small, flexible tube (bronchoscope) that's passed through your mouth or nose into your lungs. The risks of bronchoscopy are generally minor — most often a temporary sore throat and hoarseness from the bronchoscope — but the tissue samples are sometimes too small for an accurate diagnosis.

Bronchoalveolar lavage. In this procedure, your doctor injects about a tablespoon of salt water through a bronchoscope into a section of your lung, and then immediately suctions it out. The solution that's withdrawn contains cells from your air sacs. Although bronchoalveolar lavage samples a larger area of the lung than other procedures do, it may not provide enough information to diagnose pulmonary fibrosis.

Surgical biopsy. Although this is a more invasive procedure with potential complications, it's often the only way to obtain a large enough tissue sample to make an accurate diagnosis. While you are under general anesthesia, surgical instruments and a small camera are inserted through two or three small incisions between your ribs. The camera allows your surgeon to view your lungs on a video monitor while removing tissue samples from your lungs.

Treatment Option

The lung scarring that occurs in interstitial lung disease can't be reversed, and treatment will not always be effective in stopping the ultimate progression of the disease. Some treatments may improve symptoms temporarily or slow the disease's progress. Others help improve quality of life.

Because many of the different types of scarring disorders have no approved or proven therapies, clinical studies may be an option to receive an experimental treatment.

Medications

Intense research to identify treatment options for specific types of interstitial lung disease is ongoing. Based on currently available, scientific evidence, however, your doctor may recommend:

Corticosteroid medications. Many people diagnosed with interstitial lung diseases are initially treated with a corticosteroid (prednisone), sometimes in combination with other drugs that suppress the immune system. Depending on the cause of the interstitial lung disease, this combination may slow or even stabilize disease progression.

Medications that slow the progression of idiopathic pulmonary fibrosis. The medications pirfenidone (Esbriet) and nintedanib (Ofev) may slow the rate of disease progression. Treatment-related side effects may be significant. Talk through the pros and cons of these medications with your doctor.

Medications that reduce stomach acid. Gastroesophageal reflux disease (GERD) affects the majority of people with idiopathic pulmonary fibrosis and is associated with worsening lung damage. If you have symptoms of acid reflux, your doctor may prescribe GERD therapies that reduce stomach acid, including H-2-receptor antagonists or proton pump inhibitors such as lansoprazole (Prevacid 24HR), omeprazole (Prilosec OTC) and pantoprazole (protonix).

Oxygen therapy

Using oxygen can't stop lung damage, but it can:

Make breathing and exercise easier

Prevent or lessen complications from low blood oxygen levels

Reduce blood pressure in the right side of your heart

Improve your sleep and sense of well-being

You're most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.

Pulmonary rehabilitation

The aim of pulmonary rehabilitation is not only to improve daily functioning but also to help people with intersitial lung disease live full, satisfying lives. To that end, pulmonary rehabilitation programs focus on:

Physical exercise, to improve your endurance

Breathing techniques that improve lung efficiency

Emotional support

Nutritional counseling

Surgery

Lung transplantation may be an option of last resort for some people with severe interstitial lung disease who haven't benefited from other treatment options.

Lifestyle and home remedies

Being actively involved in your own treatment and staying as healthy as possible are essential to living with interstitial lung disease. For that reason, it's important to:

Stop smoking. If you have lung disease, the best thing you can do for yourself is to stop smoking. Talk to your doctor about options for quitting, including smoking cessation programs, which use a variety of proven techniques to help people quit. And because secondhand smoke also can be harmful to your lungs, don't allow people to smoke around you.

Eat well. People with lung disease may lose weight both because it's uncomfortable to eat and because of the extra energy it takes to breathe. These people need a nutritionally rich diet that contains adequate calories. A dietitian can give you further guidelines for healthy eating.

Get vaccinated. Respiratory infections can worsen symptoms of intersitial lung disease. Make sure you receive the pneumonia vaccine and an annual flu shot.

Coping and support

Living with a chronic lung disease is emotionally and physically challenging. Your daily routines and activities may need to be adjusted, sometimes radically, as breathing problems worsen or health care needs take priority in your life. Feelings of fear, anger and sadness are normal as you grieve for the loss of your old life and worry about what's next for you and your family.

Share your feelings with your loved ones and your doctor. Talking openly may help you and your loved ones cope with the emotional challenges of your disease. In addition, clear communication will help you and your family plan effectively for your needs if your disease progresses.

You may also want to consider joining a support group, where you can talk to people who are facing challenges similar to yours. Group members may share coping strategies, exchange information about new treatments or simply listen as you express your feelings. If a group isn't for you, you may wish to talk with a counselor in a one-on-one setting.

Preparing for your appointment

You'll probably first bring your symptoms to the attention of your family doctor. He or she may refer you to a pulmonologist — a doctor who specializes in lung disorders. Testing generally includes a variety of blood tests, a CT scan of the chest and pulmonary function testing.

What you can do

Before your appointment, you might want to write a list that answers the following questions:

What are your symptoms and when did they start?

Are you receiving treatment for any other medical conditions?

What medications and supplements have you taken in the past five years, including over-the-counter medications or illicit drugs?

What are all the occupations you've ever had, even if only for a few months?

Do any members of your family have a chronic lung disease of any kind?

Have you ever received chemotherapy or radiation treatments for cancer?

Do you have any other medical conditions, especially arthritis?

If your primary care physician had a chest X-ray done as part of your initial evaluation, bring that with you when you see a pulmonologist. It will help the pulmonologist make a diagnosis if he or she can compare an old chest X-ray with the results of a current X-ray.

The actual X-ray image is more important to your doctor than is the report alone. CT scans of your chest also may have been done, and those should also be requested.

What to expect from your doctor

Your doctor may ask some of the following questions:

Are your symptoms persistent, or do they seem to disappear and then reappear?

Have you recently had new contact with the following: air conditioners, humidifiers, pools, hot tubs, or water-damaged walls or carpet?

Are you exposed to mold or dust in your home or other homes where you spend a lot of time?

Have any close relatives or friends been diagnosed with a related condition?

Do you come into contact with birds through your work or hobbies? Does a neighbor raise pigeons?

Does your work history include regular exposure to toxins and pollutants, such as asbestos, silica dust or grain dust?

Do you have any family history of lung disease?

Do you or did you smoke? If so, how much? If not, have you spent a lot of time around others who smoke?

Have you been diagnosed or treated for any other medical conditions?

Do you have symptoms of gastroesophageal reflux disease (GERD), such as heartburn?

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