Pulmonary fibrosis

Pulmonary fibrosis

Description, Causes and Risk Factors:

Pulmonary fibrosis is a serious disease that causes progressive scarring of your lung tissue. The current thinking is that pulmonary fibrosis begins with repeated injury to the tissue within and between the tiny air sacs (alveoli) in your lungs. The damage eventually leads to scarring (fibrosis), which stiffens your lungs and makes breathing difficult. Although the exact incidence and prevalence of the disease is not known, patients with this disease comprise about 15% of a pulmonary physician's practice.

Causes of pulmonary fibrosis

Hundreds of factors can cause the lung damage that eventually leads to pulmonary fibrosis. Some of the most common include:

    Occupational and environmental factors: Long-term exposure to a number of toxins and pollutants can damage your lungs. Among them are silica dust (silicosis) and asbestos fibers (asbestosis). Chronic exposure to some organic substances, including grain dust, sugar cane, and bird and animal droppings, also can cause fibrosis.

  • Radiation: A small percentage of people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment. The severity of the damage depends on how much of the lung is exposed to radiation, the total amount of radiation administered, whether chemotherapy also is used and the presence of underlying lung disease.

  • Medications: Many drugs can damage your lungs, especially chemotherapy drugs; medications used to treat heart arrhythmias and other cardiovascular problems; certain psychiatric medications; and some antibiotics.

  • Gastroesophageal reflux disease (GERD): Acid reflux, which occurs when stomach acids back up into your food pipe (esophagus), appears to play a significant role in pulmonary fibrosis. Although people with pulmonary fibrosis frequently have GERD, they may not have typical GERD symptoms, such as heartburn and belching.

  • Other medical conditions: Serious lung infections such as tuberculosis and pneumonia can cause permanent lung damage. So can disorders that affect tissue throughout your body, not just your lungs, such as systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, polymyositis, Sjogren's syndrome and sarcoidosis. In rare cases, scleroderma is associated with a particularly severe form of pulmonary fibrosis.

Researchers have several theories about what might trigger idiopathic pulmonary fibrosis, including viruses and exposure to tobacco smoke. And because one type of idiopathic pulmonary fibrosis runs in families, heredity also is thought to play a role, even in people who don't directly inherit the disease.


Signs and symptoms of pulmonary fibrosis include:

    Shortness of breath (dyspnea).

  • A dry cough.

  • Fatigue.

  • Unexplained weight loss.

  • Aching muscles and joints.

  • Clubbing.

  • Loss of appetite.

With severe pulmonary fibrosis, complications may develop, such as congestive heart failure, pulmonary hypertension (high blood pressure), strokes, blood clots in the lungs (pulmonary embolism), and respiratory failure.


Diagnosing pulmonary fibrosis can be extremely challenging. The difference between idiopathic and nonidiopathic forms of the disease is not always clear, and the naming and classification systems for both have historically been confusing and controversial. In addition, many medical conditions, including chronic obstructive pulmonary disease (COPD), asthma and even heart failure, can mimic pulmonary fibrosis, so doctors must rule these out before making a definitive diagnosis.

A complete medical history, physical exam and even a chest X-ray aren't enough to diagnose pulmonary fibrosis, but they can help exclude other conditions. For that reason, you may have tests such as:

    Chest X-ray: This will usually show the scar tissue typical of pulmonary fibrosis and is useful for following the course of the illness and treatment. Occasionally, the chest X-ray is normal and further tests are required to explain the unexplained shortness of breath (dyspnea).

  • High-resolution computerized tomography (HRCT) scan:This provides sharper and more-detailed images of your lungs than do conventional CT scans or regular chest X-rays.

  • Pulmonary function tests: These tests determine how well your lungs work overall. They measure how much air your lungs can hold, and how quickly you can move air in and out of your lungs.

  • Oximetry:This simple test uses a small device placed on one of your fingers to measure the oxygen saturation in your blood. Oximetry can serve as an easy way to monitor the course of the disease, sometimes more accurately than a chest X-ray can.

  • Exercise stress test: An exercise test on a treadmill or stationary bike may be used to monitor your lung function when you're active.

Often, though, 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 (transbronchial biopsy): 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 sore throat and temporary hoarseness from swallowing the bronchoscope — but the tissue samples are sometimes too small for an accurate diagnosis.

  • Bronchoalveolar lavage: In this procedure, your doctor injects salt water (saline) 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 (video-assisted thoracoscopic surgery):In some cases, your doctor may recommend a 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. During the procedure, 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. Because video-assisted thoracoscopic surgery doesn't require cutting through a rib, you're likely to have less pain and to heal more quickly than you are with traditional open-lung surgery.


Specific pulmonary fibrosis treatment options include:


  • Oxygen therapy.

  • Pulmonary rehabilitation.

  • Lung transplantation.

The specific plan a healthcare provider recommends will be based on several factors, such as the person's age, other existing medical conditions he or she may have, and how much damage has already occurred.

Regardless of the treatment for pulmonary fibrosis that is recommended, it is extremely important that people quit smoking. It is also important to understand that in some people, treatment can slow down the speed at which the condition progresses; in other cases, it will not have an impact.

Medicines used in pulmonary fibrosis treatment:

It is important to treat pulmonary fibrosis as early as possible. By addressing the condition before a lot of scarring has taken place, it may improve the chances that treatment will be successful and increase the life expectancy in some people.

The mainstay of pulmonary fibrosis treatment is medication that helps to reduce inflammation. Inflammation is thought to play a role in the injury and fibrosis of the lung tissue. Therefore, medications that stop the inflammation are thought to have an effect on the resulting damage.

The antiinflammatory medicine that most healthcare providers prescribe for pulmonary fibrosis is high-dose prednisone. Prednisone is part of a group of medications known as glucocorticoids, corticosteroids, or simply "steroids" for short. People who have pulmonary fibrosis usually take prednisone by mouth every day.

However, because prednisone can cause serious side effects and does not work in many cases, a healthcare provider may only prescribe it for three to six months. Then, if improvement is seen, he or she may reduce the dose over time and keep the person on it for a longer period (this is known as a maintenance dose).

Immune Suppressants: Many healthcare providers prescribe a second medicine along with prednisone, such as azathioprine (Imuran®) or cyclophosphamide (Cytoxan®). These drugs can be prescribed alone if a person does not respond well to prednisone or develops serious side effects.

Azathioprine is a medicine that affects the immune system. Most people take it by mouth every day. Because it can cause serious side effects, a healthcare provider may prescribe it with prednisone for only three to six months. Then, if the person doesn't have serious side effects and the drug combination seems to help, the healthcare provider may prescribe it for a longer period.

Cyclophosphamide is another immune system suppressant that healthcare providers use to treat pulmonary fibrosis. They usually prescribe it in addition to low doses of prednisone for patients who are getting worse while only taking prednisone. Many patients who can't take prednisone can take cyclophosphamide by itself.

Most people take cyclophosphamide by mouth every day. Some people with pulmonary fibrosis receive it for three to five days through a needle that is inserted into a vein in the arm. After that, they take it by mouth every day. Patients usually start on a low dose, which is then increased over time. It may take three to six months before any benefits are seen. Also, there are serious cyclophosphamide side effects.

Other medicines that your healthcare provider may recommend when treating pulmonary fibrosis include:

    The influenza vaccine (the flu shot) and pneumonia vaccine to help prevent infections.

  • Cough medicines or oral codeine, which may relieve coughing.

  • Vitamin D, calcium, and osteoporosis medications to prevent bone loss if you are taking prednisone or another corticosteroid.

  • Medication for GERD.

Side effects of medications usually present. Risks and benefits of medications must be discussed with your doctor.

Oxygen Therapy: Oxygen therapy is often recommended to help decrease shortness of breath and allow a person to be more active. Early on in the disease, oxygen may only be necessary with exercise or sleep. However, as the condition worsens, treatment with oxygen may be necessary all of the time.

Pulmonary Rehabilitation: Pulmonary rehabilitation has become the standard of care for anyone with a chronic lung disease, including pulmonary fibrosis. This treatment method allows people to better manage their condition and improve their quality of life. Services often include:

    Breathing exercises and retraining so that it takes less energy to breathe.

  • Physical conditioning training.

  • Anxiety, stress, and depression management.

  • Nutritional counseling.

  • Support groups.

Lung Transplantation: Lung transplantation is an option for some people diagnosed with pulmonary fibrosis. This is more often an option for people under the age of 65, who are not benefiting from medicines, and who have no other medical problems.

Early referral for a lung transplant evaluation is important. It is also important to understand that while a lung transplant can improve the quality of life and life expectancy, it is a major operation with serious risks.

Side effects of drugs are positive. Discuss risks and benefits of medications before taking any medications.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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