Bay Biosciences provides high-quality biopsy tissue samples, formalin fixed paraffin embedded (FFPE) tissue blocks, with matched fresh frozen sera (serum), plasma, and peripheral blood mononuclear cells (PBMC) bio-fluids, from patients diagnosed with idiopathic pulmonary fibrosis (IPF).

The sera (serum), plasma and peripheral blood mononuclear cells (PBMC) biofluid specimens are processed from patients peripheral whole-blood using customized collection and processing protocols from idiopathic pulmonary fibrosis (IPF) patients.

Fresh frozen tissue and matched biofluid samples were, collected from unique patients diagnosed with idiopathic pulmonary fibrosis (IPF).

Bio-samples are provided to a valued pharmaceutical customer for research, diagnostics, discovery, and drug development.

Idiopathic Pulmonary Fibrosis (IPF) Overview

Idiopathic pulmonary fibrosis (IPF) is a scarring of the lungs, it is a chronic, progressive lung disease. This condition causes scar tissue (fibrosis) to build up in the lungs, which makes the lungs unable to transport oxygen into the bloodstream effectively.

The disease usually affects people between the ages of 50 and 70. Idiopathic pulmonary fibrosis (IPF) belongs to a group of conditions called interstitial lung diseases (also known as ILD), which describes lung diseases that involve inflammation or scarring in the lung.

The word “idiopathic” means unexplained or unknown. Doctors use it when they are not able to identify the exact cause of a disease or a condition . That’s the case with idiopathic pulmonary fibrosis (IPF).
Researchers believe some things you may have breathed in could be linked to idiopathic pulmonary fibrosis (IPF). For example, if you spend time working around metal or wood dust, or asbestos, you may be at higher risk for the disease.
Whatever the cause, idiopathic pulmonary fibrosis (IPF) leads to scarring in your lungs, which makes your airways thick and stiff. It becomes harder and harder to breathe.

According to the national heart lung, and blood institute (NHLBI), idiopathic pulmonary fibrosis (IPF) is a progressive condition with no known cure. However, treatment plans can help people manage the condition and improve their quality of life.

Idiopathic pulmonary fibrosis (IPF) is a serious chronic disease that affects the tissue surrounding the air sacs, or alveoli, in your lungs. This condition occurs when that lung tissue becomes thick and stiff for unknown reasons.

Over time, these changes can cause permanent scarring in the lungs, called fibrosis, that make it progressively more difficult to breathe.

According to the Pulmonary Fibrosis Foundation, 50,000 new cases of IPF are diagnosed each year in the United States.

In the past, other names for idiopathic pulmonary fibrosis (IPF) included cryptogenic fibrosing alveolitis, idiopathic diffuse interstitial pulmonary fibrosis, and idiopathic fibrosing alveolitis, chronic form. These days, some doctors may refer to idiopathic pulmonary fibrosis (IPF) as usual interstitial pneumonia.


Types of Pulmonary Fibrosis

According to the American Lung Foundation, there are more than 200 types of pulmonary fibrosis (PF), and the most common is IPF.

The Pulmonary Fibrosis Foundation state that there are five main categories for PF based on their causes:

  • Drug-induced PF: Due to the previous or current use of drugs that affect the lungs, such as chemotherapy, amiodarone (Pacerone), or nitrofurantoin (Macrobid).
  • Radiation-induced PF: Due to radiation treatment of the chest.
  • Environmental PF: Due to exposure from lung irritants, such as mold.
  • Autoimmune PF: Due to autoimmune diseases, such as rheumatoid arthritis (RA) or Sjögren’s Syndrome.
  • Occupational PF: Due to exposure to dust or fumes, such as asbestos or silica.

IPF differs from the other form of PF because it has no known cause.

Stages of Idiopathic Pulmonary Fibrosis (IPF)

The authors of a 2014 article state that there are no official stages of idiopathic pulmonary fibrosis (IPF) but that doctors have traditionally loosely staged IPF using terms such as early or advanced, or mild, moderate, or severe.

Mild Idiopathic Pulmonary Fibrosis (IPF)

At this stage of IPF patients may show mild symptoms of idiopathic pulmonary fibrosis (IPF) and receive an initial diagnosis.

In some cases, though, patients may not have any symptoms until the moderate stage of idiopathic pulmonary fibrosis (IPF).

Moderate Idiopathic Pulmonary Fibrosis (IPF)

At this stage, there will be a large amount of scarring in the lungs, and patients may need oxygen therapy.

Advanced Idiopathic Pulmonary Fibrosis (IPF)

Symptoms are severe at this stage, and a lung transplant may be necessary.

Signs and Symptoms of Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) causes scarring and stiffness in the lungs. Over time, the scarring will become severe, and the stiffness will make breathing harder. the symptoms of IPF develop gradually over time for some people but appear more quickly for others.

Following are some of the symptoms of idiopathic pulmonary fibrosis (IPF):

The course of pulmonary fibrosis, and the severity of symptoms can vary considerably from patient to patient. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen more slowly, over months or years.

Some idiopathic pulmonary fibrosis (IPF) patients may experience a rapid worsening of their symptoms (acute exacerbation), such as severe shortness of breath, that may last for several days to weeks.

Eventually, the lungs may not be able to take in enough oxygen to supply the body with the amount it needs. This can ultimately lead to respiratory failure, heart failure, and other health problems.

IPF patients who have acute exacerbations may have to be placed on a mechanical ventilator. Doctors may also prescribe antibiotics, corticosteroid medications or other medications to treat an acute exacerbation.


Causes of Idiopathic Pulmonary Fibrosis (IPF)

Pulmonary fibrosis scars thickens the tissue around and between the air sacs (alveoli) in the lungs. This makes it more difficult for oxygen to pass into the bloodstream.

The damage can be caused by many different factors, including long-term exposure to certain toxins, certain medical conditions, radiation therapy and some medications.

The National Organization for Rare Disorders (NORD) suggest that most cases of IPF occur as a result of damage to the epithelial cells that line the alveoli and airways.

The NORD suggest that the body attempts to repair the damaged cells, but the response is abnormal, resulting in progressive damage and scarring to the lung tissue and alveoli.

However, researchers do not know why the initial damage that triggers the healing response occurs.

Although there is no known cause of IPF, according to the National Library of Medicine, researchers think that a mix of environmental, lifestyle, and genetic factors play a part in the disease.

The most significant risk factor appears to be a variation in the MUC5B gene, which results in high mucus production in the respiratory bronchioles.

Doctors may look at several potential causes when diagnosing pulmonary fibrosis. These can include:

However, in the majority of pulmonary pulmonary fibrosis, doctors aren’t able to identify a cause. If they’re unable to find a cause, the disease will be labeled idiopathic.

Genetic factors may play some role in the development of pulmonary fibrosis and idiopathic pulmonary fibrosis (IPF), but it’s rare for these conditions to occur in more than one member of the same family.

Occupational and Environmental Factors

Long-term exposure to certain toxins and pollutants can damage the lungs. These include the following:

  • Asbestos fibers 
  • Bird and animal droppings 
  • Coal dust
  • Grain dust
  • Hard metal dusts
  • Silica dust

Radiation Treatments

Some patients 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 may depend on:

  • How much of the lung was exposed to radiation
  • The total amount of radiation administered
  • Whether chemotherapy also was used
  • The presence of underlying lung diseases

Medications

Many drugs can damage your lungs, especially medications such as the following:

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

Medical Conditions

Lung damage can also result from a number of conditions, including the following:

Many substances and conditions can lead to pulmonary fibrosis. Even so, in most cases, the cause is never found. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis (IPF).

Researchers have several theories about what might trigger idiopathic pulmonary fibrosis (IPF), including viruses and exposure to tobacco smoke. Also, some forms of idiopathic pulmonary fibrosis (IPF) run in families, and heredity may play a role in idiopathic pulmonary fibrosis (IPF).

Many people with idiopathic pulmonary fibrosis (IPF) may also have gastroesophageal reflux disease (GERD), which is a condition that occurs when acid from your stomach flows back into your esophagus.

Ongoing research is evaluating if GERD may be a risk factor for idiopathic pulmonary fibrosis, or if GERD may lead to a more rapid progression of the condition. However, more research is needed to determine the association between idiopathic pulmonary fibrosis (IPF) and GERD.

Risk Factors of Idiopathic Pulmonary Fibrosis (IPF)

Following are the risk factors of idiopathic pulmonary fibrosis (IPF):

  • Genetics: Patients who have a family history of idiopathic pulmonary fibrosis (IPF) have a higher risk of IPF, particularly if a first degree relative, such as a parent or sibling, has IPF. However, IPF can also occur sporadically.
  • Smoking cigarettes: About 75% of patients with IPF are smokers or have previously been smokers.
  • Gastroesophageal reflux disease (GERD): Roughly 75% of people with IPF have acid reflux  or heartburn symptoms.
  • Being male: Approximately 75% of patients with IPF are male.
  • Age: Most patients with IPF are over the age of 50 years.

Diagnosis of Idiopathic Pulmonary Fibrosis (IPF)

Since the signs and symptoms of IPF develop slowly over time, it’s difficult for doctors to diagnose idiopathic pulmonary fibrosis (IPF) immediately. Scarring caused by IPF also looks similar to scarring caused by other conditions and lung diseases.

Several tests can be used to confirm idiopathic pulmonary fibrosis (IPF) and rule out other possible causes.

The Canadian Lung Association state that to diagnose idiopathic pulmonary fibrosis (IPF), a doctor will assess any symptoms, take a full medical history, and carry out a physical examination.

During the physical examination, the doctors may check whether the patient has:

  • Blue hands and feet
  • Clubbed toes or fingers
  • High pitched crackles coming from the lungs

A combination of tests may help a doctor confirm a diagnosis of idiopathic pulmonary fibrosis (IPF).

These may include the following tests:

  • Blood Tests: Blood works are performed to check and identify reasons for lung scarring. Blood gas test measures the blood levels of oxygen and carbon dioxide.
  • Chest X-ray: To look for signs of disease and progression.
  • CT Scan: To identify scarring in the lungs.
  • Exercise Tests: To test the function of the lungs.
  • High-resolution computer tomography (HRCT) scan.
  • Lung Biopsy: To confirm a diagnosis if this is not possible based on a CT scan.
  • Lung Function Test (LFT): This breathing test  is performed to check how well the lungs breathe air in and out and how well they oxygenate the blood. 
  • Pulse oximetry: Pulse oximetry measures the oxygen levels in the blood.

 

Treatment of Idiopathic Pulmonary Fibrosis (IPF)

According to the NHLBI currently there is no cure for idiopathic pulmonary fibrosis (IPF), although treatments may help slow the progression of IPF and improve quality of life.

The main goals of medical treatment are to reduce lung inflammation, protect lung tissue, and slow the loss of lung function. This will  help patients breathe easier.

The most common treatment options include oxygen therapy and medications. Oxygen therapy helps with breathing, and medications help to control inflammation and reduce lung tissue scarring.

The Food and Drug Administration (FDA) has approved two IPF medications, nintedanib (Ofev) and pirfenidone (Esbriet).

The treatment options depend on the stage of idiopathic pulmonary fibrosis (IPF) but may include the following:

  • Kinase inhibitors: This type of medication can help slow down a loss of lung function and may prevent a sudden worsening of IPF.
  • Antacids: These help treat GERD if a patient also have this condition.
  • Oxygen therapy: Initially, an IPF patient may need this therapy after exertion. In the later stages of IPF, they may need it continually.
  • Ventilator support: An IPF patient may need this support if their breathing problems become severe.
  • Lung transplant: This surgery may be necessary for patients who have developed advanced forms of  IPF disease. Because it’s a major surgical procedure, a lung transplant is often seen as the final treatment step. It’s currently the only cure for IPF.

Other forms of treatment depend on the patients symptoms. For example, if a patient person has developed a lung infection, they may require to use antibiotics. For a chronic cough, they may take oral codeine.

Pulmonary rehabilitation may also be a part of their treatment plan.

Pulmonary rehabilitation is a program that may involve the following:

Complications of Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) disease may cause patients anxiety, stress, and depression. A doctor may recommend the following treatments for these conditions:

  • A patients support group
  • Cognitive behavioral therapy (CBT)
  • Antidepressant drugs

According to a article in the medical sciences journal possible complications of IPF can include:

  • Difficulty sleeping
  • Lung cancer
  • Pulmonary hypertension, which is an increase of blood pressure in the blood vessels to the lungs
  • Respiratory failure, when the lungs cannot provide enough oxygen to the blood.

 

Managing Idiopathic Pulmonary Fibrosis (IPF)

To manage idiopathic pulmonary fibrosis (IPF) it is important to adopt healthy lifestyle practices that can reduce the disease symptoms and improve the overall outlook and quality of life of the patient. Following are the recommended lifestyle changes:

  • Maintain a moderate body weight, overweight patients should loose weight
  • Quit smoking
  • Regular use and staying on top of all vaccines, medications, and vitamins or supplements
  • Using an oxygen monitor to keep your oxygen saturation (also known as blood oxygen levels) in optimal range

Idiopathic pulmonary fibrosis (IPF) patients may also consider joining a pulmonary rehabilitation support group. These groups, which may be arranged by your doctor’s office or a local hospital that you are treated, connect you with healthcare professionals and possibly other patients who are living with IPF.

Learning tools for stress management might help prevent worsening symptoms. Support groups can also ease anxiety and feelings of loneliness as you face questions or uncertainty when the disease progresses.

Together, patients can learn breathing and conditioning exercises as well as techniques for managing stress, anxiety, and the many emotions this diagnosis may cause.


Outlook for Idiopathic Pulmonary Fibrosis (IPF) Patients

Idiopathic pulmonary fibrosis (IPF) is a progressive disease, which means it will get worse over time. While  steps can be taken to manage the symptoms, the scarring and lung damage cannot be stopped entirely.

For some patients, the IPF disease may progress very quickly. For others, it may take many years before breathing problems become so difficult that they require to use oxygen.

When lung function becomes severely limited, it can trigger serious complications. These complications can include the following:

The hardened lung tissue that IPF causes may lead to respiratory failure, heart failure, or another life threatening disease.

Existing symptoms can also suddenly get worse after an infection, heart failure, or a pulmonary embolism.

Transplant surgery can help improve your outlook. Other treatments and lifestyle changes can also help slow disease progression.

Life Expectancy for Idiopathic Pulmonary Fibrosis (IPF) Patients

According to Medline Plus idiopathic pulmonary fibrosis (IPF) typically affects people between the ages of 50 and 70 years old. Because it affects people later in life, the average life expectancy after a diagnosis is 3 to 5 years.

In patients with idiopathic pulmonary fibrosis (IPF) their life expectancy will depend on several factors. These include the following:

  • Age
  • How quickly the disease progresses
  • The intensity of symptoms
  • Overall health

 

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Samples available include cancer (tumor) tissue, cancer serum, cancer plasma, cancer, peripheral blood mononuclear cells (PBMC). and human tissue samples from most other therapeutic areas and diseases.

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Bay Biosciences banks wide variety of human tissue samples and biological samples, including cryogenically preserved at – 80°C.

Including fresh frozen tissue samples, tumor tissue samples, formalin-fixed paraffin-embedded (FFPE), tissue slides, with matching human bio-fluids, whole blood and blood-derived products such as serum, plasma and PBMC.

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