What is interstitial lung disease

Interstitial lung disease is an umbrella term for a large group of disorders that includes more than 130 disorders that cause scarring (fibrosis) and/or inflammation of the lungs ¹, ². The inflammation and scarring cause stiffness in the lungs which makes it difficult to breathe and hard to get enough oxygen. The scarring is called pulmonary fibrosis.

Breathing in dust or other particles in the air is responsible for some types of interstitial lung diseases. Specific types include:

• Idiopathic Pulmonary Fibrosis
• Hypersensitivity pneumonitis
• Sarcoidosis
• Asbestosis, from inhaling asbestos fibers
• Black lung disease among coal miners, from inhaling coal dust (coal worker’s pneumoconiosis)
• Farmer’s lung, from inhaling farm dust
• Siderosis, from inhaling iron from mines or welding fumes
• Silicosis, from inhaling silica dust
• Connective tissue or autoimmune disease-related interstitial lung disease
• Wegener’s granulomatosis
• Churg Strauss (vasculitis)
• Chronic eosinophilic pneumonia
• Eosinophilic granuloma (Langerhan’s cell histoiocytosis)
• Familial Pulmonary Fibrosis
• Drug-induced lung disease
• Bronchiolitis obliterans
• Lymphangioleiomyomatosis

Interstitial lung diseases can be caused by exposure to hazardous chemicals, certain medications and medical treatments. Other causes include autoimmune diseases or occupational exposures to molds, gases, or fumes. In most cases of interstitial lung disease, the causes are unknown. Lung damage from many interstitial lung diseases is irreversible and progressive, meaning it gets worse over time. Once lung scarring occurs, it’s generally irreversible. In some cases, it can be slowed by certain medications, but many people never regain full use of their lungs. Lung transplant is an option for some people who have interstitial lung disease.

The progression of interstitial lung disease varies from disease to disease and from person to person. It is important to determine the specific form of interstitial lung disease in each person because what happens over time and the treatment may differ depending on the cause. Importantly, each person responds differently to treatment, so close monitoring during treatment is important.

Treatment depends on the type of exposure and the stage of the interstitial lung disease. It may involve medicines and oxygen therapy. Occasionally, people with interstitial lung diseases will be recommended for lung transplants.

Interstitial lung disease causes

Interstitial lung disease seems to occur when an injury to your lungs triggers an abnormal healing response. Ordinarily, your body generates just the right amount of tissue to repair damage. But in interstitial lung disease, the repair process goes awry and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into your bloodstream.

Interstitial lung disease can be triggered by many different things — including airborne toxins in the workplace, drugs and some types of medical treatments. In most cases, the causes are unknown.

Occupational and environmental factors

Long-term exposure to a number of toxins and pollutants can damage your lungs. These may include:

• Silica dust
• Asbestos fibers
• Grain dust
• Bird and animal droppings
• Radiation treatments
• Indoor hot tubs

Some people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment.

Medications

Many drugs can damage your lungs, especially:

• Chemotherapy drugs. Drugs designed to kill cancer cells, such as methotrexate (Otrexup, Trexall, others) and cyclophosphamide, can also damage lung tissue.
• Heart medications. Some drugs used to treat irregular heartbeats, such as amiodarone (Nexterone, Pacerone) or propranolol (Inderal, Innopran), may harm lung tissue.
• Some antibiotics. Nitrofurantoin (Macrobid, Macrodantin, others) and ethambutol (Myambutol) can cause lung damage.
• Anti-inflammatory drugs. Certain anti-inflammatory drugs, such as rituximab (Rituxan) or sulfasalazine (Azulfidine), can cause lung damage.

Medical conditions

Lung damage can also result from connective and autoimmune diseases such as:

• Rheumatoid arthritis
• Scleroderma
• Dermatomyositis and polymyositis
• Mixed connective tissue disease
• Vasculitis
• Systemic Lupus Erythematosus
• Sjogren’s syndrome
• Sarcoidosis
• Inherited disorders (e.g. familial pulmonary fibrosis, Hermansky-Pudlak syndrome) ³
• Primary disorders (e.g. pulmonary Langerhans cell histiocytosis) ³
• Hypersensitivity pneumonitis

The list of substances and conditions that can lead to interstitial lung disease is long. Even so, in some cases, the causes are never found. Disorders without a known cause are grouped together under the label of idiopathic interstitial pneumonias, the most common and deadly of which is idiopathic pulmonary fibrosis.

Risk factors for interstitial lung disease

Factors that may make you more susceptible to interstitial lung disease include:

• Age. Interstitial lung disease is much more likely to affect adults, although infants and children sometimes develop the disorder.
• Exposure to occupational and environmental toxins. If you work in mining, farming or construction or for any reason are exposed to pollutants known to damage your lungs, your risk of interstitial lung disease is increased.
• Gastroesophageal reflux disease (GERD). If you have uncontrolled acid reflux or indigestion, you may be at increased risk of interstitial lung disease.
• Smoking. Some forms of interstitial lung disease are more likely to occur in people with a history of smoking, and active smoking may make the condition worse, especially if there is associated emphysema.
• Radiation and chemotherapy. Having radiation treatments to your chest or using some chemotherapy drugs makes it more likely that you’ll develop lung disease.

Interstitial lung disease symptoms

The most common symptoms of interstitial lung disease are shortness of breath with exercise and a non-productive cough.

• At first, shortness of breath may not be severe and is only noticed with exercise, climbing stairs, and other activities.
• Over time, it can occur with less strenuous activity such as bathing or dressing, and as the disease worsens, even with eating or talking.
• Most people with interstitial lung disease also have a dry cough. A dry cough means you do not cough up any mucus or sputum.

These symptoms are generally slowly progressive, although rapid worsening can also occur. Some people also may have a variety of other symptoms. They may include: fever, weight loss, fatigue, muscle and joint pain and abnormal chest sounds, depending upon the cause.

Over time, weight loss, fatigue, and muscle and joint pain are also present.

People with more advanced interstitial lung disease may have:

• Abnormal enlargement of the base of the fingernails (clubbing)
• Blue color of the lips, skin, or fingernails due to low blood oxygen levels (cyanosis)
• Symptoms of the other diseases such as arthritis or trouble swallowing (scleroderma), associated with interstitial lung disease

By the time symptoms appear, irreversible lung damage has often already occurred. Nevertheless, it’s important to see your doctor at the first sign of breathing problems. Many conditions other than interstitial lung disease can affect your lungs, and getting an early and accurate diagnosis is important for proper treatment.

Interstitial lung disease complications

Interstitial lung disease can lead to a series of life-threatening complications, including:

• High blood pressure in your lungs (pulmonary hypertension). Unlike systemic high blood pressure, this condition affects only the arteries in your lungs. It begins when scar tissue or low oxygen levels restrict the smallest blood vessels, limiting blood flow in your lungs. This in turn raises pressure within the pulmonary arteries. Pulmonary hypertension is a serious illness that becomes progressively worse.
• Right-sided heart failure (cor pulmonale). This serious condition occurs when your heart’s lower right chamber (right ventricle) — which is less muscular than the left — has to pump harder than usual to move blood through obstructed pulmonary arteries. Eventually the right ventricle fails from the extra strain. This is often a consequence of pulmonary hypertension.
• Respiratory failure. In the end stage of chronic interstitial lung disease, respiratory failure occurs when severely low blood oxygen levels along with rising pressures in the pulmonary arteries and the right ventricle cause heart failure.

Stages of interstitial lung disease

In determining the progression and advancement of your interstitial lung disease, your physician or pulmonologist will typically go through two primary methods of judging your respiratory health: the GOLD (Global Initiative for Chronic Obstructive Lung Disease) System and the BODE (Body Mass, Airflow Obstruction, Dyspnea, Exercise Capacity) Index. These two tests are designed to assess your pulmonary condition through a variety of different tests such as a spirometry assessment (or pulmonary function tests), as well as several other metrics like a 6-minute walk test, oximeter results, and arterial blood-gas analysis.

The typical breakdown of interstitial lung disease stages are as follows ⁴:

1. Mild– meaning you have 5+ years with appropriate treatment
2. Moderate– meaning you have 3-5+ years with appropriate treatment
3. Severe– meaning you have 3+ years with appropriate treatment
4. Advanced– meaning you have < 3 years with appropriate treatment

Although these are the general guidelines of interstitial lung disease staging, as with any progressive lung disease, these life expectancies are largely dependent on the individual. This means that through proper treatment, diet and exercise, it’s possible to exceed these figures while maintaining a healthy quality of life.

How do I calculate my BODE Index?

You can make an estimated calculation for your BODE Index for chronic obstructive pulmonary disease (COPD) life expectancy with a BODE Index calculator (https://reference.medscape.com/calculator/bode-index-copd). Here’s what you’ll need before you enter information into the calculator:

1. FEV1 percentage predicated after using a bronchodilator to open airways from your pulmonary function test
2. Your 6-minute walk test distance
3. Body mass index (BMI)
4. Level of dyspnea or difficulty breathing.

After you enter the information, an approximate survival prediction will show. Of course, it’s important to remember that this is a tool to help estimate life expectancy, and you need to discuss your BODE Index and chronic obstructive pulmonary disease (COPD) with your doctor.

What is the GOLD Staging System?

Many doctors use more than one way to determine your chronic obstructive pulmonary disease (COPD) stage. One common way to measure COPD stages and severity is with the GOLD staging system. The Global Initiative for Chronic Obstructive Lung Disease or GOLD came up with the GOLD system. The GOLD system uses your forced expiratory volume (FEV1) test from your pulmonary function test to categorize the severity of your COPD into stages. Simply put, FEV1 means the amount of air you can forcefully exhale in 1 second. As the severity of COPD progresses, the stage number shows how airflow is limited.

Simply put, the GOLD COPD stages break down into 4 total stages. Over time, COPD symptoms will worsen, and the stage numbers will increase.

• Stage 1: Very mild COPD with a FEV1 about 80 percent or more of normal.
• Stage 2: Moderate COPD with a FEV1 between 50 and 80 percent of normal.
• Stage 3: Severe emphysema with FEV1 between 30 and 50 percent of normal.
• Stage 4: Very severe COPD with a lower FEV1 than Stage 3, or those with Stage 3 FEV1 and low blood oxygen levels

Remember, seeing your doctor regularly to keep track of your GOLD chronic obstructive pulmonary disease (COPD) stages and other measurements, such as BODE index and pulmonary function test, is important. Being proactive in your healthcare is essential to improving and maintaining your quality of life.

Interstitial lung disease diagnosis

Identifying and determining the cause of interstitial lung disease can be challenging. A large number of disorders fall into this broad category. In addition, the signs and symptoms of a wide range of medical conditions can mimic interstitial lung disease, and doctors must rule these out before making a definitive diagnosis.

Some of the following tests may be necessary.

Six minute walk test (checks how far you can walk in 6 minutes and how many times you need to stop to catch your breath)

Laboratory tests

• Blood tests. Certain bloodwork can detect proteins, antibodies and other markers of autoimmune diseases or inflammatory responses to environmental exposures, such as those caused by molds or bird protein.

Imaging tests

• Computerized tomography (CT) scan. This imaging test is key to, and sometimes the first step in, the diagnosis of interstitial lung disease. CT scanners use a computer to combine X-ray images taken from many different angles to produce cross-sectional images of internal structures. A high-resolution CT scan can be particularly helpful in determining the extent of lung damage caused by interstitial lung disease. It can show details of the fibrosis, which can be helpful in narrowing down the diagnosis and in guiding treatment decisions.
• Echocardiogram. A sonogram for the heart, an echocardiogram uses sound waves to visualize the heart. It can produce still images of your heart’s structures, as well as videos that show how your heart is functioning. This test can evaluate the amount of pressure occurring in the right side of your heart.

Pulmonary function tests

• Spirometry and diffusion capacity. This test requires you to exhale quickly and forcefully through a tube connected to a machine that measures how much air your lungs can hold, and how quickly you can move air out of your lungs. It also measures how easily oxygen can move from the lungs into the bloodstream.
• Oximetry. This simple test uses a small device placed on one of your fingers to measure the oxygen saturation in your blood. It may be done at rest or with activity to monitor the course and severity of lung disease.

Lung tissue analysis

Often, 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. 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 temporary sore throat and hoarseness from the bronchoscope — but the tissue samples are sometimes too small for an accurate diagnosis.
• Bronchoalveolar lavage (BAL). In this procedure, your doctor injects about a tablespoon of salt water 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. 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. While you are under general anesthesia, 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.

Interstitial lung disease treatment

Treatment depends on the cause and duration of the disease. Medicines that suppress the immune system and reduce swelling in the lungs are prescribed if an autoimmune disease is causing the problem. The lung scarring that occurs in interstitial lung disease can’t be reversed, and treatment will not always be effective in stopping the ultimate progression of the disease. Some treatments may improve symptoms temporarily or slow the disease’s progress. Others help improve quality of life. For some people who have idiopathic pulmonary fibrosis, pirfenidone and nintedanib are two medicines that may be used to slow the disease. If there is no specific treatment for the condition, the aim is to make you more comfortable and support lung function:

• Quit Smoking – Not only will it actively take years off of your life, but it will make your disease symptoms significantly worse. And trust, although the symptoms of interstitial lung disease may not be bad enough for you to change now, by the time they progress to the point of being unbearable, it’ll be too late to go back.
• Get Out and Exercise– we say this one a lot but it’s important to get your blood pumping and moving through your body easier. Why? Because your blood carries your oxygen to the parts of the body that need it. Even if it’s doing standing squats in front of the TV or walking to the mailbox, start small and build-up from there. If you can keep yourself disciplined and consistent in your goals you’ll be walking marathons in no time.
• People with low blood oxygen levels will receive oxygen therapy in their home. A respiratory therapist will help you set up the oxygen. Families need to learn proper oxygen storage and safety.
• Change Your Diet – it doesn’t have to be as extreme as never eating meat again and going vegetarian. Start with portions. Try to eat more grains (rice and pasta) than you eat meat. From there drop the pasta and add in more vegetables. If you want to eat dessert, swap that out with some good and well-prepared fruit.

Because many of the different types of scarring disorders have no approved or proven therapies, clinical studies may be an option to receive an experimental treatment.

Table 1. Management of the patient with interstitial lung disease or idiopathic pulmonary fibrosis

▪ Establish a partnership with the patient to provide a patient-centered, personalized medicine care plan	▪ Provide supplemental oxygen if indicated (keep SpO2 ≥90%)
▪ Provide patients with:	· During exertion
	· Nocturnal during sleep
• Useful information concerning   the nature of their disease   and its prognosis	· Continuous if indicated
• Treatment options   accompanied by   thoughtful counseling	▪ Detect and treat co-morbidities and complications:
· Enrollment in clinical trials	· Gastroesophageal reflux    disease
· Off-label therapies    (e.g. corticosteroids,    cytoxic drugs, other agents)	· Cardiovascular disease
· Lung transplantation	· Drug toxicity (if treated)
· Best supportive care	· Sleep-disordered breathing
▪ Use disease-specific monitoring (for prognostication and treatment decisions)	· Secondary pulmonary    hypertension
· Pulmonary function testing    (FVC, DLCO, 6-MWT)	· Metabolic bone disease    (osteopenia, osteoporosis)
· Thoracic imaging	· Anemia
· Dyspnea score	· Anxiety & depression
▪ Pulmonary rehabilitation (optimal exercise program, patient education)	▪ Maintain ideal body-mass index (weight reduction if obese, improved nutrition if cachectic)
	▪ Vaccinations (pneumococcal vaccine, seasonal influenza, others as indicated)

Abbreviations: DL_CO = diffusion capacity of the lung for CO; FVC = forced vital capacity; 6-MWT = six-minute walk test; SpO2 = oxyhemoglobin percent saturation.

[Source ³ ]

Table 2. Therapies for select types of interstitial lung disease

Interstitial lung disease type	Key features of immunopathogenesis	Current therapy*	Additional and/or alternative therapies
Idiopathic pulmonary fibrosis	• Prominent fibroblast proliferation and matrix deposition	Supportive care	Anti-reflux therapy
	• Patchy, temporally heterogeneous changes	Consider anti-reflux measures	N-acetylcysteine
	• Architectural distortion of tissue	– Anti-reflux surgery	Clinical trials
	• Epithelial injury, microvascular remodeling	– Acid suppressants (e.g. proton-pump inhibitor)	(experimental)
	• Variable inflammatory component (usually minimal/mild)	Pirfenidone (not approved in US)
	• Areas of non-specific interstitial pneumonia- and desquamative interstitial pneumonia-like change often present
	• Pulmonary hypertension frequently present with advanced disease	Lung transplantation
Sarcoidosis	• Well-formed non-caseating granulomata in tissues	Observation (mild/stable disease)	Infliximab
	• Extra-pulmonary disease may be present		Other immunosuppression agent
	• May be asymptomatic; may resolve spontaneously without therapy	Corticosteroids (oral or inhaled)	Lung transplantation
		Methotrexate
Non-specific interstitial pneumonia	• Homogeneous, diffuse involvement of the lung	Corticosteroids	Other immunosuppression drugs
	• Histopathologic subtypes include cellular (prominent lymphocyte influx; best prognosis), mixed (cellular & fibrotic), and fibrotic (worst prognosis)	Mycophenolate	Lung transplantation
	• Usually responsive to immunosuppression (less likely to respond if advanced fibrosis is established)
Cryptogenic organizing pneumonia	• Prominent inflammatory cell infiltrate (↑ lymphocytes, neutrophils, and/or eosinophils can all be present)	Corticosteroids	Other immunosuppression drugs
			Macrolides
	• Usually responds to immunosuppression therapy; relapse frequently occurs
Hypersensitivity pneumonitis	• Prominent lymphocyte influx with formation of loose granulomata	Exposure cessation	Other immunosuppression drugs
	• Can have appearance of cellular non-specific interstitial pneumonia or organizing pneumonia	Corticosteroids	Lung transplantation
	• Can progress to advanced fibrosis (and masquerade as idiopathic pulmonary fibrosis or fibrotic non-specific interstitial pneumonia)
Eosinophilic pneumonia	• Prominent influx of eosinophils	Corticosteroids	Other immunosuppression drugs
	• Usually responsive to immunosuppression therapy
Connective tissue disease-associated interstitial lung disease	• Lung histopathology can reveal non-specific interstitial pneumonia (common), usual interstitial pneumonia (less common); other interstitial lung disease (e.g. organizing pneumonia, desquamative interstitial pneumonia, respiratory bronchiolitis with interstitial lung disease – very uncommon)	Corticosteroids	Anti-reflux therapy
		Mycophenolate	Lung transplantation
	• Pulmonary hypertension often present (with or without interstitial lung disease)	Other disease-modifying anti-rheumatic drug agent(s)	Treatment of pulmonary hypertension
Acute interstitial pneumonia/diffuse alveolar damage	• Intense inflammation and alveolar damage	Corticosteroids	Cytotoxic drugs
	• Hyaline membrane formation
	• Prominent neutrophil influx early

Footnote: *Therapies that are usually administered on the basis of expert opinion and clinical trial results; none have received US Food and Drug Administration approval for the indication of interstitial lung disease/idiopathic pulmonary fibrosis (but pirfenidone is approved for treatment of idiopathic pulmonary fibrosis in some countries, and many DMARD (disease-modifying anti-rheumatic drug) agents are approved for treatment of connective tissue disease).

[Source ³ ]

Medications

Intense research to identify treatment options for specific types of interstitial lung disease is ongoing. Based on currently available, scientific evidence, however, your doctor may recommend:

• Corticosteroid medications. Many people diagnosed with interstitial lung diseases are initially treated with a corticosteroid (prednisone), sometimes in combination with other drugs that suppress the immune system. Depending on the cause of the interstitial lung disease, this combination may slow or even stabilize disease progression.
• Medications that slow the progression of idiopathic pulmonary fibrosis. The medications pirfenidone (Esbriet) and nintedanib (Ofev) may slow the rate of disease progression. Treatment-related side effects may be significant. Talk through the pros and cons of these medications with your doctor.
• Medications that reduce stomach acid. Gastroesophageal reflux disease (GERD) affects the majority of people with idiopathic pulmonary fibrosis and is associated with worsening lung damage. If you have symptoms of acid reflux, your doctor may prescribe GERD therapies that reduce stomach acid, including H-2-receptor antagonists or proton pump inhibitors such as lansoprazole (Prevacid 24HR), omeprazole (Prilosec OTC) and pantoprazole (protonix).

Oxygen therapy

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

• Make breathing and exercise easier
• Prevent or lessen complications from low blood oxygen levels
• Reduce blood pressure in the right side of your heart
• Improve your sleep and sense of well-being

You’re most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.

Pulmonary rehabilitation

The aim of pulmonary rehabilitation is not only to improve daily functioning but also to help people with intersitial lung disease live full, satisfying lives. To that end, pulmonary rehabilitation programs focus on:

• Physical exercise, to improve your endurance
• Breathing techniques that improve lung efficiency
• Emotional support
• Nutritional counseling

Surgery

Lung transplantation may be an option of last resort for some people with severe interstitial lung disease who haven’t benefited from other treatment options.

Lifestyle and home remedies

Being actively involved in your own treatment and staying as healthy as possible are essential to living with interstitial lung disease. For that reason, it’s important to:

• Stop smoking. If you have lung disease, the best thing you can do for yourself is to stop smoking. Talk to your doctor about options for quitting, including smoking cessation programs, which use a variety of proven techniques to help people quit. And because secondhand smoke also can be harmful to your lungs, don’t allow people to smoke around you.
• Eat well. People with lung disease may lose weight both because it’s uncomfortable to eat and because of the extra energy it takes to breathe. These people need a nutritionally rich diet that contains adequate calories. A dietitian can give you further guidelines for healthy eating.
• Get vaccinated. Respiratory infections can worsen symptoms of intersitial lung disease. Make sure you receive the pneumonia vaccine and an annual flu shot.

Coping and support

Living with a chronic lung disease is emotionally and physically challenging. Your daily routines and activities may need to be adjusted, sometimes radically, as breathing problems worsen or health care needs take priority in your life. Feelings of fear, anger and sadness are normal as you grieve for the loss of your old life and worry about what’s next for you and your family.

Share your feelings with your loved ones and your doctor. Talking openly may help you and your loved ones cope with the emotional challenges of your disease. In addition, clear communication will help you and your family plan effectively for your needs if your disease progresses.

You may also want to consider joining a support group, where you can talk to people who are facing challenges similar to yours. Group members may share coping strategies, exchange information about new treatments or simply listen as you express your feelings. If a group isn’t for you, you may wish to talk with a counselor in a one-on-one setting.

Interstitial lung disease prognosis

The chance of recovering or interstitial lung disease getting worse depends on the cause and how severe the disease was when it was first diagnosed.

Some people with interstitial lung disease develop heart failure and high blood pressure in the blood vessels of their lungs.

In the case of interstitial lung disease, the prognosis largely depends on the treatment regimen as well as cause of the disease’s development, but generally you can expect the following developments in your health:

• Fatigue
• Cyanosis
• Weight loss
• Acute pneumonia
• Shortness of breath
• Weight loss
• Abnormal enlargement of the fingernail base
• Dry, persistent cough
• High blood pressure (in some cases)
• Heart failure (in some cases)

Idiopathic pulmonary fibrosis has a poor outlook.

Interstitial lung disease survival rate

Lung transplantation is an accepted form of treatment for patients with interstitial lung disease that is progressive, clearly leading to respiratory failure, and refractory to other therapies ⁵. The number of lung transplants performed for patients with idiopathic pulmonary fibrosis surpassed that for chronic obstructive pulmonary disease (COPD) in 2007, when idiopathic pulmonary fibrosis became the leading indication for lung transplants performed in the United States ⁶. Lung transplantation is the only form of therapy that may improve quality of life and survival for patients with idiopathic pulmonary fibrosis, and 5-year survival following lung transplantation for idiopathic pulmonary fibrosis or other forms of pulmonary fibrosis is approximately 50% ⁷.

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