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About PF

About PF


Feeling confused or overwhelmed about pulmonary fibrosis? You are not alone.

The Pulmonary Fibrosis Foundation is here to help you understand what it means to have pulmonary fibrosis. You can always reach us through our Patient Communication Center at 844.Talk.PFF or by email at pcc@pulmonaryfibrosis.org.

Please remember that this information is a brief overview and is for educational purposes only. It is not intended to be a substitute for professional medical advice. Always consult your health care provider with any questions you may have regarding your specific medical condition.

What is Pulmonary Fibrosis?

The word "pulmonary" means “lung” and the word "fibrosis" means scar tissue – similar to scars that you may have on your skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. But, pulmonary fibrosis is more serious than just having a scar in your lung. In PF, the scar tissue builds up in the walls of the air sacs of the lungs, and eventually the scar tissue makes it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising.

Also, pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike (see “Causes and Symptoms” below). The PF family of lung diseases falls into an even larger group of diseases called the “interstitial lung diseases.” Some interstitial lung diseases don't include scar tissue. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.

Causes and Symptoms

It can be challenging to figure out what caused your pulmonary fibrosis. Sometimes doctors are able to identify one or more causes of your disease, but it is also common to end up without an answer, despite completing a large number of medical tests. Diseases where a specific cause is not identified are often called "idiopathic" - meaning the cause is not identified or the disease occurs spontaneously.

There are many forms of pulmonary fibrosis that are idiopathic. The one that many people have heard of is “idiopathic pulmonary fibrosis” or IPF, but there are many others. A few examples are:

  • Idiopathic nonspecific interstitial pneumonia (NSIP)
  • Cryptogenic organizing pneumonia (COP)
  • Sarcoidosis

When the cause of PF is known, if often falls into one of these categories:

Autoimmune diseases

Autoimmune diseases are also called connective tissue diseases, collagen vascular diseases, or rheumatologic diseases. “Auto” means “self” and “immune” refers to your immune system. With autoimmune diseases, your own immune system is attacking your lung. If you have an autoimmune disease, your immune system can cause inflammation and scarring in the lungs. Examples of autoimmune diseases that can cause PF include:

  • Rheumatoid arthritis
  • Scleroderma (now called “systemic sclerosis”)
  • Certain muscle diseases (polymyositis, dermatomyositis, and the anti-synthetase syndrome)

Occupational exposures

Pulmonary fibrosis can develop after significant exposure to a wide variety of inorganic dusts, including asbestos, silica, coal dust, beryllium, hard metal dusts.

Environmental exposures 

Organic dusts, including animal proteins, bacteria, and molds, can also contribute to the development of some types of PF. Diseases caused by inhaled organic dusts are often called “hypersensitivity pneumonitis”. Exposure to radiation can also contribute to the risk of PF.

Drug Induced or Medication exposures

Some medications have been linked to the development of PF, including drugs used to treat:
  • Infections (nitrofurantoin, sulfasalazine)
  • Heart disease (amiodarone, propranolol)
  • Seizures (phenytoin)
  • Cancer (methotrexate, bleomycin, oxaliplatin, radiation therapy)

Genetic/Inherited diseases

Approximately 10-15% of those with an “idiopathic” form of PF have another family member afflicted by the disease. This is called familial pulmonary fibrosis (FPF) or familial interstitial pneumonia (FIP). A number of genes and genetic variants have been identified that are associated with the development pulmonary fibrosis, but frequently genetic tests are not performed when PF is diagnosed, as we are only just beginning to understand the significance of these genetic abnormalities. It is important to discuss the potential risks and benefits of genetic testing with a qualified genetic counselor and your medical provider. To speak with a Certified Genetic Counselor free of charge, contact Janet Talbert, MS, CGC at 800.423.8891, extension 1097.

CLASSIFICATION OF COMMON INTERSTITIAL LUNG DISEASES


Symptoms

The most common symptoms of PF are cough and shortness of breath. Symptoms may be mild or even absent early in the disease process. As the lungs develop more scar tissue, symptoms worsen. Shortness of breath initially occurs with exercise, but as the disease progresses patients may become breathless while taking part in everyday activities, such as showering, getting dressed, speaking on the phone, or even eating.

Due to a lack of oxygen in the blood, some people with idiopathic pulmonary fibrosis may also have “clubbing” of the fingertips. Clubbing is a thickening of the flesh under the fingernails, causing the nails to curve downward. It is not specific to IPF and occurs in other diseases of the lungs, heart, and liver, and can also be present at birth.

Other common symptoms of pulmonary fibrosis include:

  • Chronic dry, hacking cough
  • Fatigue and weakness
  • Discomfort in the chest
  • Loss of appetite
  • Unexplained weight loss

Diagnostics and Tests

Interstitial lung diseases (ILD) are a group of disorders that affect the lung tissue around the air sacks and airways. Many types of ILD can lead to scaring of the lungs or PF. Making an accurate diagnosis of ILD is complicated by the fact that there are more than 200 different types. In fact, one study showed that more than half of people with IPF may have been initially misdiagnosed. The Pulmonary Fibrosis Foundation strongly recommends that everyone with suspected or established PF be seen at least once at a center that specializes in the care of people with PF. To find medical care, click here.

If possible, it is very important to determine which type of pulmonary fibrosis you have. Distinct types of PF behave differently in terms of how quickly your disease will progress, and how you may respond to different treatments prescribed by your medical professional.

In order to determine the type of PF you have, doctors from a variety of specialties, including pulmonologists (respirologists), radiologists, rheumatologists, and pathologists work together to make an accurate diagnosis. Your medical provider(s) will order a variety of tests; some of the most common tests are discussed below.

Comprehensive Medical History

It is essential for your medical provider to obtain a comprehensive medical history to learn if any medications, environmental/occupational exposures, familial, or other medical conditions could have contributed to your pulmonary fibrosis.

Physical Examination

It is important to undergo an in-depth physical examination. Scar tissue can often be heard as "crackles" or Velcro-like sounds when your medical provider listens to your lungs with a stethoscope. Your provider may also detect signs of other medical conditions that could be affecting your lungs.

Upon reviewing your medical history and physical examination, your medical provider may recommend a variety of tests to further diagnose your lung disease.

Most people with PF will also undergo one or more of the following tests during their evaluation:

High-Resolution Computed Tomography (HRCT) scan of the chest

A type of x-ray test that allows your provider to get a detailed look at your lungs and is a critical component of the diagnostic evaluation of PF.

Blood Tests

Your medical provider may also perform a series of blood tests to see if your immune system is contributing to your lung disease.

Pulmonary Function Tests

Pulmonary Function Tests (PFTs) measure the size of your lungs, how well air moves in and out of your lungs, and how well your lungs bring oxygen into your body.

These tests help your medical provider determine the type and severity of lung disease present. They are also used to monitor for response to therapy and disease progression. 
PFTs are usually performed in a hospital or clinical laboratory and consist of breathing through a "spirometer." Sometimes they are also performed while you sit inside a large plastic enclosure that looks like a glass telephone booth.

Pulse Oximetry

A “pulse oximeter” is a device placed on the fingertip or earlobe that indicates the amount of oxygen in the blood. Normally, nearly all of your red blood cells are full of oxygen. When all the red blood cells are carrying oxygen, the blood is called “100% saturated.” A normal saturation level is between 95% - 100% when the test is performed at sea level. Low oxygen saturation values can help your medical professional identify the presence of lung disease. Early on in PF, oxygen levels may be normal while you are resting, but can decrease while exerting yourself. This drop in oxygen saturation (called “de-saturation”) can make you feel breathless. When oxygen levels drop below 89% during exertion, your medical professional may recommend using supplemental oxygen while active.  

Arterial Blood Gas

Arterial Blood Gas (ABG) is a direct arterial puncture to measure arterial pH, oxygen saturation (PaO2), and carbon dioxide content (PCO2). Arterial blood has been oxygenated by the lungs and thus indicates how much oxygen is available to the body.

Bronchoscopy

Bronchoscopy is a procedure used to collect samples of cells and small biopsies from your lungs using a bronchoscope. Examination of these samples can be useful in making a diagnosis of certain types of lung diseases.

Bronchoalveolar Lavage

Bronchoalveolar Lavage (BAL) is a way to remove a tiny sampling of cells from the lower respiratory tract using a bronchoscope. During this test, a small amount of saline is injected through the bronchoscope and, when withdrawn, removes a sample of cells from the respiratory tract. Frequently, this test is not helpful in making the diagnosis of PF, but may be beneficial in other clinical situations.

Exercise Testing

Exercise testing is a measure of how well the lungs function during exertion. Test methods vary from hospital to hospital, but frequently include the use of a stationary bike or treadmill. The most common method of exercise testing is the six-minute walk test, where the distance you can walk in six minutes is measured. Blood pressure, electrocardiogram, and oxygen saturation levels may also be monitored during exercise testing.

Esophagram

Esophagram is an X-ray examination of the esophagus (the tube that carries food to your stomach). This exam will help to determine if you suffer from gastroesophageal reflux disease (GERD) or other disorders of the esophagus, which can accompany certain forms of PF.

Echocardiogram 

Echocardiogram (ECHO) is a test that uses sound waves (ultrasound) to create a picture of your heart. It provides information about your heart function and screens for the presence of pulmonary hypertension, a condition that can accompany PF. 

Surgical Lung Biospy

In some cases a biopsy of the lung is required to make a diagnosis of PF. A biopsy should only be considered after an extensive evaluation. Usually a lung biopsy can be performed by a minimally invasive procedure called a video assisted thoracoscopic surgery, or VATS. A VATS procedure is usually well tolerated, but it may not be recommended for all patients. If your medical provider recommends a lung biopsy, he/she will discuss the recommended procedure with you and refer you to a thoracic surgeon.

GLOSSARY

  • Alveoli: Tiny air sacs in the lungs where carbon dioxide leaves the bloodstream and oxygen enters the bloodstream.
  • Bronchoscope: An instrument used for inspecting the airways of the lungs.
  • Comorbidity: A disease or other medical issue that occurs simultaneously with PF.
  • Diffuse parenchymal lung diseases (DPLD): Another name for interstitial lung diseases.
  • Diffusion capacity (DLCO): A measure of the ability of oxygen to diffuse into the bloodstream.
  • Dyspnea: Difficulty breathing or shortness of breath.

    Exacerbation: An episode of rapid decline or the emergence of more severe symptoms.
  • Fibroproliferation:  Relating to the growth of fibroblasts (and fibrous tissue), one of the basic connective tissue cells.
  • Fibrosis: An increase in fibrous scar tissue.
  • Forced expiratory volume (FEV1): The amount of air you can blow out in one second, which is measured by spirometry.
  • Forced vital capacity (FVC): How much air you can blow out of your lungs, which is measured by spirometry.
  • Gastroesophageal reflux disease (GERD): A regurgitation of stomach acids into the esophagus and throat, causing heartburn, acid indigestion, and possibly injury to the lining of the esophagus. This is also called acid reflux disease.
  • Hospice care: Palliative care for patients at end-of-life.
  • Idiopathic: Of unknown cause.
  • Idiopathic interstitial pneumonias (IIP): A type of interstitial lung disease. Idiopathic pulmonary fibrosis is a type of IIP.
  • Interstitial lung diseases (ILD): A broad category of over 200 lung diseases that affect the lung interstitium.
  • Interstitium: The space between the alveoli (air sacs).
  • Palliative care: Non-curative therapy that treats symptoms and focuses on improving quality of life. It can be received at the same time as curative therapy.
  • Pathologist: A physician specializing in disease-associated changes in tissue and organs. Pathologists aid in making a medical diagnosis.
  • Pulmonary: Relating to the lungs.
  • Pulmonary hypertension: Abnormally high blood pressure in the pulmonary (lung) arteries.
  • Pulmonologist: A physician specializing in the lungs.
  • Radiologist: A physician specializing in using radiology tests(e.g., X-rays) to diagnose illness.
  • Rheumatologist: A physician specializing in rheumatic diseases, which may include arthritis, autoimmune diseases, and joint diseases.
  • Spirometry: A test that measures the amount of air inhaled and exhaled over time.
  • Usual interstitial pneumonia (UIP): A specific abnormal radiologic or pathologic pattern of interstitial lung disease.

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