Overview

Pulmonary fibrosis is an illness of the lung which occurs when lung tissue gets damaged. The thickened, stiffer tissue makes it difficult for your lungs to function efficiently. As the severity of pulmonary fibrosis increases, it becomes increasingly tired.

A myriad of reasons drives the scarring caused by pulmonary fibrosis. In most cases, doctors don’t know the cause of the problem. If a cause isn’t determined, the problem is idiopathic pulmonary fibrosis.

The damage to the lungs due to pulmonary fibrosis cannot be fixed; however, therapies and medicines can relieve symptoms and improve the quality of life. For certain people, an organ transplant may be the best option.

Signs and symptoms of pulmonary fibrosis

The signs or symptoms that are indicative of fibrosis could include:

  • Breathlessness (dyspnea)
  • Dry cough
  • Fatigue
  • Unexplained weight loss
  • Joints and muscles that are achy
  • The widening and rounding of fingers’ tips (or toes) (clubbing)

Pulmonary fibrosis course and the degree of symptoms will vary from one person to the next. Some sufferers develop the disease quickly, with severe illness. Other sufferers experience mild symptoms, which slowly worsen over time or many years.

Certain people might notice a rapid increase in its signs (acute exacerbation), such as intense shortness of breath that may last for days or weeks. Individuals who suffer from acute exacerbations might be put on mechanical ventilators. In addition, doctors can prescribe antibiotics, corticosteroid medications, or other medicines for treating acute exacerbations.

The Reasons

The fibrosis in your lungs causes scars and thickens the tissues between and around the air sacs (alveoli) inside your lung, making it harder for oxygen to enter the bloodstream. The harm can be caused by many causes, including prolonged exposure to toxic chemicals and medical ailments, radiation therapy, and certain drugs.

Environmental and occupational factors

Excessive exposure to a range of pollutants and toxins can cause damage to the lungs of a person. This includes:

  • Silica dust
  • Asbestos fibers
  • Hard metal dust
  • Coal dust
  • Grain dust
  • Drops of animal and bird waste

Based on the American Lung Association the majority of people suffering from IPF suffer from pulmonary fibrosis.

Treatments with radiation

Certain patients who undergo radiation treatment for breast or lung cancer may show signs of damage to the lung several months or even years following the treatment. The extent of the damage can depend on:

  • What percentage of the lung was exposed
  • The total amount of radiation absorbed.
  • If chemotherapy was also employed
  • The presence of lung disease

Medicines

Many medications can cause damage to your lungs, particularly drugs like:

  • Chemotherapy medicines. Medications designed to eliminate cancerous cells, like methotrexate (Trexall, Otrexup, others) and cyclophosphamide, also harm lung tissue.
  • Heart medication. Some medicines used to treat irregular heartbeats like Amiodarone (Cordarone, Nexterone, Pacerone) could cause damage to lung tissue.
  • Certain antibiotics. Antibiotics such as Nitrofurantoin (Macrobid Macrodantin), Macrobid, etc.) or ethambutol may cause lung damage.
  • Anti-inflammatory medications. Certain anti-inflammatory drugs like rituximab (Rituxan) or Sulfasalazine (Azulfidine) may cause lung damage.

Medical medical

Lung damage can also arise from a range of factors which include:

  • Dermatomyositis
  • Polymyositis
  • A disorder of connective tissue is called mixed connective tissue.
  • Systemic Lupus Erythematosus
  • Rheumatoid arthritis
  • Sarcoidosis
  • Scleroderma
  • Pneumonia

Numerous conditions and chemicals can trigger pulmonary fibrosis. However, in most instances, the root cause is not identified. Pulmonary fibrosis that has no reason is referred to as idiopathic pulmonary fibrosis.

Researchers have many theories regarding the triggers for idiopathic pulmonary inflammation, which could be caused by viruses or smoking tobacco. In addition, certain types of idiopathic lung fibrosis are passed through families, and heredity could contribute to Idiopathic pulmonary fibrosis.

Many people suffering from idiopathic pulmonary inflammation may also have gastroesophageal reflux disorder (GERD). This illness develops in the stomach, where stomach acid is reabsorbed into the esophagus. Research is currently evaluating whether GERD might be an underlying cause of Idiopathic pulmonary fibrosis or the possibility that GERD can cause a greater rate of development of the disease. But, more research is required to find out the relationship between idiopathic lung fibrosis and GERD.

Risk factors

Factors that can make you more prone to pulmonary fibrosis may include:

  • Age. Although pulmonary fibrosis is a condition that has been identified in infants and children, however, it is more likely to be affecting older and middle-aged adults.
  • Sexually. Idiopathic pulmonary fibrosis is more likely to affect males than women.
  • Smoking Habit. Far more smokers and former smokers suffer from lung fibrosis than those who have never been smokers. Pulmonary fibrosis is a possibility in people suffering from Emphysema.
  • Certain professions. You have an increased chance of developing pulmonary fistula if you work in agriculture, mining, or construction. You may also be exposed to chemicals known to cause lung damage.
  • Cancer treatments. Having radiation treatments to your chest or taking certain chemotherapy medications can increase your risk of developing pulmonary fibrosis.
  • Genetic causes. Some types of pulmonary fibrosis are passed down through families, and genetic causes could be the cause.

Complications

Complications of pulmonary fibrosis can include:

  • High blood pressure is present in the lung (pulmonary hypertension). Unlike systemic high blood pressure, this issue only affects the arteries of your lung. It is triggered with the smallest streets, and capillaries are compressed due to scar tissue, increasing pressure on blood vessels within the lung.
  • It increases pressure within the pulmonary vessels and the upper right-hand chamber (right ventricle). There are various forms of pulmonary hypertension. They are serious diseases that get gradually more severe and may be fatal.
  • Right-sided heart defect (cor the pulmonale). This serious problem occurs when the heart’s lower right chamber (ventricle) is forced to be pumped more than usual to circulate blood through pulmonary arteries that are partially blocked.
  • Reflux failing. It is often the final stage of chronic lung disease. It happens when blood oxygen levels are at a dangerously low level.
  • Lung cancer. Long-standing pulmonary fibrosis can increase your chances of developing lung cancer.
  • Complications of the lungs. As pulmonary fibrosis develops, it can result in complications like blood clots within the lungs, collapsed lungs, or a lung infection.

Diagnosis

To determine the cause of your illness, Your doctor will review the medical and family history, check the signs and symptoms you’ve experienced and the exposure you’ve had to gas, dust, or chemicals, then perform an examination. During your physical exam, your physician will employ a stethoscope to observe the lungs of your breathing. The doctor might also recommend one or several of the tests listed below.

Imaging tests

  • Chest Xray. A chest X-ray depicts pictures from your chest. It could reveal the scar tissue common to lung fibrosis. It can be beneficial in monitoring the progression of the disease and for treatment. Sometimes, however, the chest X-ray could be normal and additional tests could be needed to understand the reason for your breathlessness.
  • The computer-generated tomography (CT) scan. CT scanners use computers to blend the X-ray images from different angles to create cross-sectional pictures of internal structures within the body. A High-resolution CT scan is useful in determining the severity of lung damage caused by fibrosis. Additionally, certain types of fibrosis exhibit distinct patterns.
  • Echocardiogram. An echocardiogram uses sound waves to show the heart. It can produce still images of the heart’s structure and videos that demonstrate how your heart works. The test will determine the pressure present on the right-hand side of your heart.

Lung function tests

  • Tests for pulmonary function. Several types of tests for pulmonary function can be performed. For a test known as spirometry, the patient exhales rapidly and vigorously through a tube connected to the machine. The machine determines how much air your lungs can hold and the speed at which you transfer air into and out of your lung. Other tests can assess your lung volume and capacity for diffusing.
  • Pulse Oximetry. This simple test utilizes a tiny device put on one of your fingers to gauge the level of oxygen in your blood. Oximetry can be used as an indicator of the progression of the illness.
  • Stress test for exercise. An exercise test using stationary bikes or a treadmill can be used to check your lung function while you’re engaged.
  • Blood gas tests for arterial blood. In this test, the doctor examines an amount of your blood, typically from an artery located in your wrist. The carbon dioxide and oxygen levels of the blood sample are later measured.

Treatment

The lung scarring that happens in pulmonary fibrosis cannot be removed. Currently, no treatment has proven to be effective in stopping the condition’s progression. Certain medicines can ease symptoms temporarily or even slow down the progress of the disease. Other treatments can improve the quality of life. Doctors will assess the severity of the illness to determine the best treatment for your condition.

Medical Treatment

A physician may suggest newer treatments, such as pirfenidone (Esbriet) and Nintedanib (Ofev). Which medicines can help to slow down the progress of idiopathic fibrosis? Both therapies were approved by Food and Drug Administration (FDA). New formulations and additional medications of these medicines are in development but are not yet FDA accepted by the FDA.

Nintedanib can trigger negative side effects like nausea and diarrhea. Some of the side effects of pirfenidone can be nausea, rash, and diarrhea.

Researchers continue to research medications for treating pulmonary fibrosis.

Doctors might recommend anti-acid medication to treat gastroesophageal respiratory disease (GERD) as an inflammatory condition typically in people suffering from an idiopathic pulmonary condition.

Oxygen therapy

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

  • Help your breathing and exercise become easier.
  • Reduce or prevent complications caused by low levels of blood oxygen
  • Lower blood pressure on the right-hand side of your heart.
  • Enhance your sleep and feel of wellbeing

It is possible to receive oxygen while you are exercising or sleeping. Still, some individuals may make use of it continuously. Some people carry a cylinder of oxygen, which makes the oxygen more portable.

Rehabilitation of the pulmonary system

The rehabilitation program for your lungs can help you manage symptoms and help you improve your daily function. Rehabilitation programs in the field of pulmonary rehabilitation concentrate on:

  • Exercise to increase your endurance
  • Breathing techniques that could enhance the efficiency of your lung.
  • Counseling for nutrition
  • Support and counseling
  • Information about your illness

Lung transplant

Lung transplantation could be an option for patients suffering from pulmonary hypertrophy. The procedure can enhance your quality of life and help you enjoy a longer lifespan. However, a transplant may cause complications, like rejection and infection. Your physician may be able to consult with you about whether the idea of a lung transplant is appropriate for your particular situation.

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