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About Pulmonary Fibrosis

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Feeling confused or overwhelmed about pulmonary fibrosis or idiopathic 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 Support line at 844-825-5733 or by email 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. Over time, the scar tissue can destroy the normal lung and make 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. Pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike. The PF family of lung diseases falls into an even larger group of diseases called the interstitial lung diseases (also known as ILD), which includes all of the diseases that have inflammation and/or scarring in the lung. Some interstitial lung diseases don’t include scar tissue. When an interstitial lung disease does include scar tissue in the lung, we call it pulmonary fibrosis.

No one is certain how many people are affected by PF. One recent study estimated that idiopathic pulmonary fibrosis (or IPF, which is just one of more than 200 types of PF) affects 1 out of 200 adults over the age of 65 in the United States—that translates to more than 200,000 people living with PF today. Approximately 50,000 new cases are diagnosed each year and as many as 40,000 Americans die from IPF each year.

Causes & Symptoms of Pulmonary fibrosis 

What causes pulmonary fibrosis?

It can be challenging for doctors to figure out what causes PF. Sometimes they are able to identify one or more causes of your disease, which are discussed here. PF of unknown cause is called “idiopathic”

There are five main categories of identifiable causes of pulmonary fibrosis: Drug-induced, Radiation-induced, Environmental, Autoimmune, and Occupational. In the United States, Environmental and Autoimmune causes seem to be the most common types of PF of known cause.

This table shows some of the clues that doctors use to identify these known causes of PF.

TYPE OF PULMONARY
FIBROSIS   
CLUES THAT DOCTORS USE  
Drug-induced Prior or current use of amiodarone, nitrofurantoin, chemotherapy,     methotrexate, or other drugs known to affect the lungs
Radiation-induced Prior or current radiation treatment to the chest
Environmental (called
hypersensitivity pneumonitis)
Exposure to mold, animals, or other triggers ("squeaks" heard in the lung)
Autoimmune (called
connective tissue disease-related)
Joint inflammation, skin changes (particularly on the fingers and face), dry eyes or mouth, abnormal blood tests
Occupational (called pneumoconiosis)  Prior or current exposure to dusts, fibers, fumes, or vapors that can cause PF (such as asbestos, coal, silica, and others)

Some MEDICATIONS can cause PF. Drugs used to treat cancer (chemotherapy), drugs used to treat abnormal heart rhythms (such as amiodarone), drugs used to treat inflammatory conditions (such as methotrexate), and an antibiotic used to treat urinary tract infections (nitrofurantoin) are some of the better known drugs that can cause injury, inflammation, and scarring in the lungs. Numerous other drugs have been implicated as causes of PF in some cases.

RADIATION to the chest for lymphoma; Hodgkin’s disease; or breast, lung, and other cancers can injure the lung and cause fibrosis.

ENVIRONMENTAL CAUSES OF PF are typically called hypersensitivity pneumonitis (HP) or chronic hypersensitivity pneumonitis. HP occurs when the lungs react with inflammation and scarring after breathing in mold spores, bacteria, animal proteins (especially from indoor or caged birds), or other known triggers. No one is certain why some people are so susceptible to developing HP and others are not.

AUTOIMMUNE DISEASES are also called connective tissue diseases, collagen vascular diseases, or rheumatologic diseases. “Auto” means self and “immune” refers to your immune system. So if you have an autoimmune disease affecting your lungs, it means that your body’s immune system is attacking your lungs. Examples of autoimmune diseases that can cause PF include
  • Rheumatoid arthritis;
  • Scleroderma (also called systemic sclerosis);
  • Sjögren’s syndrome; and
  • Polymyositis, dermatomyositis, and antisynthetase syndrome.
OCCUPATIONAL CAUSES OF PF, also called pneumoconiosis, can develop after significant exposure to a wide variety of inorganic dusts, including asbestos, silica, coal dust, beryllium, and hard metal dusts.

Are there genetic or inherited forms of pulmonary fibrosis?

Yes, but in most cases, it is not as straightforward as inheriting blue eyes or red hair. There are three different scenarios:

1) Did the genes I inherited from my parents contribute to PF even if I am the only one in my family with PF?

Yes. The risk of developing most human diseases is influenced by the genes you inherited from your parents. There have been several genes identified that appear to increase the risk of developing PF when an abnormal form of the gene is inherited. There are no official medical guidelines on whether or not genetic testing should be performed if you have PF, although in some cases, your doctor might wish to test you for one or more abnormal forms of these genes.

2) Can PF run in the family?

Yes. Anywhere from three to 20% of people with PF have another family member with PF. But this doesn’t mean that up to 20% of your family members will develop PF. In most cases, the chance that one of your family members will develop PF is very low. We encourage you to talk to your doctor or to a genetic counselor about the chances that one of your family members will develop PF.

3) Are there specific types of PF where the entire disease is due to one gene? Yes. There are a few rare genetic forms of PF that can affect both children and adults when just a single gene is inherited in an abnormal form (usually from both parents). Hermansky-Pudlak syndrome is an example of a genetic form of PF that simply requires abnormalities in one gene. People living with Hermansky-Pudlak syndrome often also have very light skin pigmentation (due to low levels of melanin in their skin) and bleeding problems (due to poorly functioning platelets in their blood). Dyskeratosis congenita is another example of a genetic form of PF that can be accompanied by poorly growing fingernails, changes in skin pigmentation, increased risks of developing problems in the bone marrow, and other conditions. 

Idiopathic Pulmonary Fibrosis

My doctor said my disease was “idiopathic.” What does that mean?

Even after an exhaustive evaluation, doctors sometimes end up telling their patients, “We don’t know why you have this.” Often, the word “idiopathic” is used to refer to any disease of unknown cause. One type of idiopathic lung disease that you may have heard of is idiopathic pulmonary fibrosis (IPF), but there are actually many other forms of ILD that are idiopathic.

IDIOPATHIC PULMONARY FIBROSIS (IPF) is a scarring disease of the lungs of unknown cause. To make a diagnosis of IPF, your doctor will perform a thorough history to try to identify potential exposures or other diseases that might lead to scarring of the lung. If a plausible cause is found, then you do not have IPF. The scarring pattern of IPF is technically called usual interstitial pneumonia (UIP). Your doctor will use detailed X-rays of your lungs called high-resolution computed tomography (HRCT) and sometimes a lung biopsy to look for this pattern. A diagnosis of IPF requires that your doctor cannot find a cause and the presence of a pattern of UIP on either HRCT or a surgical lung biopsy sample.

Although IPF is still considered to be a disease of unknown cause, we do know some factors that increase the risk of getting IPF, including aging (IPF is rare before age 50), cigarette smoking, and having certain genetic predispositions.

Symptoms

The most common symptoms of PF are dry, hacking 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:

  • Fatigue and weakness
  • Discomfort in the chest
  • Loss of appetite
  • Unexplained weight loss

Diagnosis

 How do doctors recognize and diagnose pulmonary fibrosis? There are three consequences of PF. Doctors use these consequences to recognize that someone has PF.

  • STIFF LUNGS— Scar tissue and inflammation make your lungs stiff. Stiff lungs are hard to stretch, so your breathing muscles have to work extra hard just to pull air in with each breath. Your brain senses this extra work, and it lets you know there’s a problem by triggering a feeling of breathlessness(or “shortness” of breath) while exerting yourself. Also, stiff lungs hold less air (they shrink up a bit). Doctors take advantage of this “shrinking” to diagnose and track the disease using breathing tests (called Pulmonary Function Tests) that measure how much air your lungs can hold. The more scar tissue your lungs have, the less air they will hold.
  • LOW OXYGEN LEVELS IN THE BLOOD— Scar tissue blocks the movement of oxygen from the inside of your air sacs into your bloodstream. For many people living with PF, oxygen levels are only reduced a little bit while resting, but their oxygen levels drop quite a bit during exertion. The brain can sense these low oxygen levels, triggering breathlessness. Doctors will check your oxygen levels to see if they drop after walking, a clue that PF might be present. Doctors also often prescribe oxygen to be used through a nasal cannula or a facemask during exertion and sleep for those with PF. As PF progresses, oxygen may be needed 24 hours a day.
  • “CRACKLES”— Your doctor may have told you that “crackles” were heard in your lungs. Crackles (also called “rales”) sound like Velcro being pulled apart. They are heard in many lung diseases because any type of problem affecting the air sacs (such as PF, pneumonia, or a buildup of fluid in the lungs from heart failure) can cause crackles. Some people with PF don’t have crackles, but most do.

Once your doctor recognizes that you might have PF, the next step is to try to diagnose the specific kind of PF you have— there are more than 200 different kinds. Doctors typically start by asking many questions, performing a careful physical examination, and ordering a lot of blood tests. See Causes for more information.

A doctor will also use a special kind of X-ray of the chest, called a high-resolution computed tomography (HRCT) scan, so that they can see what your lung tissue looks like. HRCT scans give a close-up view of the lungs, providing more detail than routine CT scans (also known as CAT scans). Healthy lung tissue looks nearly black on a CT scan. Scar tissue and inflammation both appear grey or white. Many forms of PF look similar on a CT scan to the untrained eye, but subtle findings on HRCT scans are critically important when trying to identify which type of PF you have.

Sometimes, even after all of the testing is complete, a doctor will still not have an answer and will have to perform a lung biopsy to sort out which of the 200 different types of PF you have. When indicated, a lung biopsy can also help you and your doctor decide which treatments might be helpful.

Newly Diagnosed

I was just diagnosed with pulmonary fibrosis. What do I do next?

We suggest that you consider making an appointment with a pulmonologist who has experience caring for patients with PF. A knowledgeable team of PF experts will help make sure you receive an accurate diagnosis and the most up-to-date treatments and management recommendations. To assist you in identifying pulmonologists closer to home and developing expertise in the care of patients with PF, the Pulmonary Fibrosis Foundation established the PFF Care Center Network that includes 40 medical centers throughout the United States. You can find a list of sites within the Network at pulmonaryfibrosis.org/life-with-pf/find-medical-care.   

We also recommend that you consider joining a PF support group. Connecting with other individuals facing the same illness can help you and your family not feel so alone in your journey with pulmonary fibrosis. Support groups can supplement the care you receive from your health care team by providing emotional support and education.

Support groups can help those living with pulmonary fibrosis

  • learn about their disease and available treatments;
  • feel supported by others who are going through the same thing;
  • learn to navigate the health care system more effectively; and
  • improve coping skills, among other things.

You can learn more and find a local PF support group at pulmonaryfibrosis.org/life-with-pf/support-groups.

Contact the PFF Patient Communication Center (PCC) by calling 844.TalkPFF (844.825.5733) or email pcc@pulmonaryfibrosis.org with any questions or concerns you may have and to connect you with local resources.

GLOSSARY

  • Acute exacerbation: An episode of rapid worsening of a pulmonary (relating to lungs) condition.
  • Alveoli: Tiny air sacs in the lungs where carbon dioxide leaves the bloodstream and oxygen enters the bloodstream.
  • Bronchial tree: The series of airways connecting the trachea (windpipe) to the alveoli.
  • Bronchus: One of the airways of the bronchial tree (plural: bronchi).
  • Bronchoscope: A tool usually passed through the nose or mouth used for inspecting the inside of airways (bronchial tubes) of the lungs. Biopsies of the lungs can be performed by bronchoscopy.
  • Comorbidity: A disease or other medical problem that occurs simultaneously with PF. A comorbidity is typically neither a cause nor a consequence of PF.
  • Diffuse parenchymal lung diseases (DPLD): Another name for interstitial lung diseases.
  • Diffusion capacity (DLCO): A measure of the ability of gases to diffuse into the bloodstream.
  • Dyspnea: Difficulty breathing, shortness of breath, or breathlessness.
  • Exacerbation: An episode of rapid decline or the emergence of more severe symptoms.
  • Fibroproliferation:  The growth of fibroblasts, the cells that make scar tissue.
  • Fibrosis: Scar tissue.
  • Forced expiratory volume (FEV1): The amount of air you can blow out oin one second after filling up your lungs with as much air as possible. Measured by a test called spirometry.
  • Forced vital capacity (FVC): The amount of air you can blow out of your lungs seconds after filling up your lungs with as much air as possible. Measured by a test called spirometry.
  • Gastroesophageal reflux disease (GERD): A medical condition defined by passage of stomach contents into the esophagus (food pipe) and often into the throat. GERD can cause discomfort (“heartburn” or “ acid indigestion”) and sometimes injures the lining of the esophagus. Also called acid reflux disease.
  • Hospice care: Focuses on caring, not curing with an emphasis on comfort and support for patients, hospice is designated for patients with a life expectancy of 6 months or less certified by a physician.
  • Idiopathic: Of unknown cause.
  • Idiopathic interstitial pneumonias (IIP): A family of 9 types of ILD of unknown cause.
  • Interstitial lung diseases (ILD): A broad category of over 200 lung diseases that affect the lung interstitium. Typically, ILDs cause inflammation, fibrosis (scarring), or an accumulation of cells in the lung not due to infection or cancer.
  • Interstitium: The walls of the air sacs of the lung. Your lung is make of air, interstitium, and blood vessels.
  • 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, which connect the heart to the lungs.
  • 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 autoimmune diseases and joint diseases.
  • Spirometry: A test that measures the amount of air inhaled and exhaled with each breath.
  • Usual interstitial pneumonia (UIP): A specific abnormal radiologic or pathologic pattern of interstitial lung disease.

Abbreviation List

  • AIP: acute interstitial pneumonia
  • CTD-ILD: connective tissue disease interstitial lung disease
  • FPF: familial pulmonary fibrosis
  • FVC: forced vital capacity
  • GERD: gastroesophageal reflux disease
  • HRCT: high-resolution computed tomography
  • ILD: interstitial lung disease
  • IPF: idiopathic pulmonary fibrosis
  • NSIP: non-specific interstitial pneumonia
  • OSA: obstructive sleep apnea
  • PFTs: pulmonary function tests
  • RA-ILD: rheumatoid arthritis ILD
  • UIP: usual interstitial pneumonia
  • VATS: video-assisted thoracoscopy surgery
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