Pulmonary Fibrosis : A case Based Discussion

Description

• Pulmonary fibrosis is a lung disease where the tissue around the air sacs in the lungs becomes damaged.

• The most common symptom of pulmonary fibrosis is difficulty breathing.

• Most of the time, the cause of pulmonary fibrosis is not known.

• Smoking and a family history of pulmonary fibrosis can increase your risk.

What is pulmonary fibrosis?

Pulmonary fibrosis is a lung disease. The tissue around the air sacs of the lungs — known as alveoli — becomes damaged, thickened, and scarred.

As the lungs scar and stiffen, breathing becomes more difficult. It can mean that not enough oxygen enters your blood.

What are the symptoms of pulmonary fibrosis?

The symptoms of pulmonary fibrosis can include:

• being short of breath — at first only when you’re active, but then later when you’re just resting

• a dry, hacking cough that does not go away

• being tired

• losing weight

• losing your appetite

• having bulging finger or toe tips, known as clubbing

• aching joints and muscles

Pulmonary fibrosis symptoms tend to get worse with time.

What causes pulmonary fibrosis?

Most often, the cause of pulmonary fibrosis is not known. If this is the case, it is called idiopathic pulmonary fibrosis (idiopathic means the cause is unknown).

In some people, a cause can be found. Some causes include:

• certain medicines

• having radiation treatment in the past

• breathing in harmful dust or chemicals at a workplace or in the environment, like asbestos, silica, metal dusts, and coal dust

• having an autoimmune disease like rheumatoid arthritis or a connective tissue disease

• having certain inflammatory conditions, such as sarcoidosis

You are more at risk than others of developing pulmonary fibrosis if you:

• are over 60 years old

• smoke or used to smoke

• work or live in an environment where you breathe in smoke or dust from wood, metal, stone, coal or sand

• have family members who have pulmonary fibrosis.

How is pulmonary fibrosis diagnosed?

Your doctor will talk with you about your symptoms and examine you.

They may ask about your work, smoking habits, and anything else that could affect your lungs.

Your doctor may recommend tests such as:

• blood tests

• chest x-ray

• CT scan or an MRI scan

• lung function tests

• a bronchoscopy (a procedure to look inside your lungs)

• a lung biopsy, where a small sample of your lung tissue is taken to be examined in the laboratory

How is pulmonary fibrosis treated?

There is no cure for pulmonary fibrosis, but there are several treatments available. Treatments can:

• stop or slow the progression of the disease

• help improve symptoms

Treatment will depend on many things, including the cause of your pulmonary fibrosis.

Medicines and oxygen

Antifibrotic medicines are available that can slow down idiopathic pulmonary fibrosis in those with mild or moderate disease. There are 2 medicines currently available:

• nintedanib

• pirfenidone

Medicines to reduce inflammation may be helpful for some people. Medicines to control breathlessness and oxygen may also help.

Pulmonary rehabilitation

Many people benefit from pulmonary rehabilitation, which is a program that includes exercise, information, and advice to help manage breathlessness.

Other treatments

A lung transplant may be recommended for some people with severe pulmonary fibrosis.

For people with severe pulmonary fibrosis, palliative care can help control symptoms and improve quality of life.

Can pulmonary fibrosis be prevented?

Things you can do which may help prevent pulmonary fibrosis include:

• quitting smoking

• eating a healthy diet

• exercising and staying physically active

• getting treatment if you have a chest infection

• avoiding known causes and risk factors where possible

Complications of pulmonary fibrosis

Having idiopathic pulmonary fibrosis can increase your chance of developing:

• lung cancer

• pulmonary hypertension, where the blood pressure in the arteries that carry blood from the heart to the lungs is higher than normal

• lung infections

• anxiety

• depression

  
  

• Can pulmonary fibrosis be prevented?

  Things you can do which may help prevent pulmonary fibrosis include:

  • quitting smoking

  • eating a healthy diet

  • exercising and staying physically active

  • getting treatment if you have a chest infection

  • avoiding known causes and risk factors where possible

  Complications of pulmonary fibrosis

  Having idiopathic pulmonary fibrosis can increase your chance of developing:

  • lung cancer

  • pulmonary hypertension, where the blood pressure in the arteries that carry blood from the heart to the lungs is higher than normal

  • lung infections

  • anxiety

  • depression

  Talk to your doctor if you are concerned about complications or need support.

• Diagnosis

  To diagnose pulmonary fibrosis, your doctor or other healthcare professional reviews your medical and family history and does a physical exam. You can talk about your symptoms and review any medicines you take. You also will likely be asked about any continuous or repeated contact with dusts, gases, chemicals or similar substances, especially through work.

  During the physical exam, your healthcare professional listens carefully to your lungs while you breathe. Pulmonary fibrosis often occurs along with a crackling sound at the base of the lungs.

  You may have one or more of these tests.

  Imaging tests

  • Chest X-ray. Images of the chest may show the scar tissue that is usually part of pulmonary fibrosis. Sometimes the chest X-ray may not show any changes. More tests may be needed to find out why you are short of breath.

  • Computerized tomography (CT) scan. A CT scan combines X-ray images taken from many different angles to create images of structures inside the body. A high-resolution CT scan can be helpful in diagnosing pulmonary fibrosis and in finding out how much lung damage has occurred. Some kinds of fibrosis have certain patterns.

  • Echocardiogram. An echocardiogram uses sound waves to look at the heart. The test can create pictures of the heart's structures. It also can create videos that show how the heart is working. This test can tell the amount of pressure in the arteries of the lungs and in the right side of the heart.

  Lung function tests

  Also called pulmonary function tests, these are done to find out how well your lungs are working:

  • Spirometry. In this test, you breathe out quickly and forcefully through a tube connected to a machine. The machine measures how much air the lungs can hold and how quickly air moves in and out of the lungs.

  • Lung volume test. This test measures the amount of air the lungs hold at different times when breathing in and out.

  • Lung diffusion test. This test shows how well the body moves oxygen and carbon dioxide between the lungs and the blood.

  • Pulse oximetry. This simple test uses a small device placed on one of the fingers to measure how much oxygen is in the blood. The percentage of oxygen in the blood is called oxygen saturation. Your healthcare professional may recommend a six-minute walking test with a check of your oxygen saturation.

  • Exercise stress test. An exercise test on a treadmill or stationary bike may be used to monitor heart and lung function during activity.

  • Arterial blood gas test. In this test, a sample of blood, usually taken from an artery in the wrist, is tested. The oxygen and carbon dioxide levels in the sample are measured.

  In addition to showing whether you have pulmonary fibrosis, imaging and lung function tests can be used to check your condition over time and see how treatments are working.

  Tissue sample

  If other tests cannot find the cause of your condition, a small amount of lung tissue may need to be removed. This is called a biopsy. The biopsy sample is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. One of these methods can be used to get a tissue sample:

  • Surgical biopsy. Although a surgical biopsy is invasive and has potential complications, it may be the only way to make the right diagnosis. This procedure may be done as a minimally invasive surgery called video-assisted thoracoscopic surgery (VATS). The biopsy also may be done as an open surgery called a thoracotomy.

    During VATS, a surgeon inserts surgical instruments and a small camera through two or three small cuts between the ribs. The surgeon looks at the lungs on a video monitor while removing tissue samples from the lungs. During the surgery, a combination of medicines put you in a sleep-like state called general anesthesia.

    During a thoracotomy, a surgeon removes a lung tissue sample through a cut that opens the chest between the ribs. This open surgery also is done using general anesthesia.

  • Bronchoscopy. In this procedure, very small tissue samples are removed — usually no larger than the head of a pin. A small, flexible tube called a bronchoscope is passed through the mouth or nose into the lungs to remove the samples. The tissue samples are sometimes too small to make the right diagnosis. But this form of biopsy also may be used to rule out other conditions.

  Blood tests

  You may have blood tests to look at your liver and kidney function. Blood tests also can check for and rule out other conditions.

  Care at Mayo Clinic

  Our caring team of Mayo Clinic experts can help you with your pulmonary fibrosis-related health concernsStart Here

  More Information

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  Treatment

  The lung scarring and thickening that occurs in pulmonary fibrosis cannot be repaired. And no current treatment has proved effective in stopping the disease from getting worse over time. Some treatments may improve symptoms for a time or slow how fast the disease worsens. Others may help improve quality of life.

  Treatment depends on the cause of your pulmonary fibrosis. Doctors and other healthcare professionals evaluate how severe your condition is. Then together you can decide on the best treatment plan.

  Medicines

  If you have idiopathic pulmonary fibrosis, your healthcare professional may recommend the medicine pirfenidone (Esbriet) or nintedanib (Ofev). Both are approved by the U.S. Food and Drug Administration (FDA) for idiopathic pulmonary fibrosis. Nintedanib also is approved for other types of pulmonary fibrosis that get worse quickly. These medicines may help slow the worsening of pulmonary fibrosis and may prevent bouts when symptoms suddenly get worse.

  Nintedanib can cause side effects such as diarrhea and nausea. Side effects of pirfenidone include nausea, loss of appetite and skin rash from sunlight. With either medicine, your healthcare professional uses regular blood tests to check how well the liver is working.

  New medicines and therapies are being developed or tested in clinical trials but are not yet approved by the Food and Drug Administration (FDA). Researchers continue to study medicines to treat pulmonary fibrosis.

  Doctors may recommend anti-acid medicines if you have symptoms of gastroesophageal reflux disease (GERD). GERD is a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis.

  Oxygen therapy

  Using extra oxygen, called supplemental oxygen, cannot stop lung damage, but it can:

  • Make breathing and exercise easier.

  • Prevent or lessen complications from low blood oxygen levels.

  • Possibly lessen strain on the right side of the heart.

  • Improve sleep and sense of well-being.

  You may use oxygen when you sleep or exercise. But some people need oxygen all the time. Carrying a small tank of oxygen or using a portable oxygen concentrator can help you be more mobile.

  Pulmonary rehabilitation

  Pulmonary rehabilitation can help manage your symptoms and improve your ability to do daily tasks. Pulmonary rehabilitation programs focus on:

  • Physical exercise to improve how much you can do.

  • Breathing techniques that may improve how well your lungs use oxygen.

  • Nutritional counseling.

  • Emotional counseling and support.

  • Education about your condition.

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