Idiopathic Pulmonary Fibrosis (IPF) Plasma

Bay Biosciences provides high quality, clinical grade, cryogenically preserved matched K2EDTA plasma, sera (serum) and peripheral blood mononuclear cells (PBMC) biofluid samples from idiopathic pulmonary fibrosis (IPF) patients.

The K2EDTA plasma, sera (serum) and PBMC biofluid specimens are processed from idiopathic pulmonary fibrosis (IPF) patient’s peripheral whole-blood using customized collection and processing protocols.

Idiopathic Pulmonary Fibrosis (IPF) Overview

Idiopathic Pulmonary Fibrosis (IPF) is a chronic and progressive lung disease. IPF causes scar tissue to build up in the lungs, leading to shortness of breath and a persistent cough.

According to the National Heart, Lung, and Blood Institute (NHLBI), IPF is a progressive condition with no known cure. However, treatment plans can help people manage the condition and improve their quality of life.

IPF causes scar tissue to grow inside your lungs and makes it hard to breathe. It gets worse over time.

The IPF scar tissue is thick, like the scars you get on your skin after a cut. It slows oxygen flow from your lungs to your blood, which can keep your body from working as it should.

There’s no cure for IPF. For most patients, symptoms don’t get better, but treatments can slow the damage to your lungs. Everyone’s outlook is different. Some patients will get worse quickly, while others can live 10 years or more after diagnosis. There are treatments to help you breathe easier and manage your symptoms. You may be able to have a lung transplant.

According to the NHLBI IPF occurs when the lung tissue that surrounds the air sacs thickens and becomes stiff, leading to permanent scarring of the lungs. This scarring affects the function of the lungs, making it difficult to breathe.

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

Idiopathic Pulmonary Fibrosis (IPF) Signs and Symptoms

According to the NHLBI, the symptoms of IPF develop gradually over time for some people but appear more quickly for others.

The symptoms of IPF include:

A dry, hacking cough that gets worsen and doesn’t go away
Aching muscles and joints
Chest pain or tightness
Feeling short of breath, which tends to occur when exercising but may worsen over time and cause people to feel out of breath when resting
Fatigue
General feeling of being unwell
Loss of appetite
Leg swelling
Shortness of breath, especially when you walk or do other activities
Unexplained Weight Loss over time

Other symptoms of IPF include:

Feeling more tired than usual
Joint and muscle aches
Weight loss without trying
Clubbing, when the tips of your fingers and toes get wider

Idiopathic Pulmonary Fibrosis (IPF) Stages

According to a 2014 article state that there are no official stages of IPF but that doctors have traditionally loosely staged IPF using terms such as early or advanced, or mild, moderate, or severe.

Mild IPF

At this stage of IPF, people may show mild symptoms of IPF and receive an initial diagnosis.

In certain cases, though, people may not have any symptoms until the moderate stage of IPF.

Moderate IPF

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

Advanced IPF

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

Types of Idiopathic Pulmonary Fibrosis (IPF)

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 states 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 therapy 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.

Idiopathic pulmonary fibrosis (IPF) differs from the other form of pulmonary fibrosis (PF) because it has no known cause.

Causes of Idiopathic Pulmonary Fibrosis (IPF)

According to the National Organization for Rare Disorders (NORD) 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.

Other risk factors for IPF include:

Genetics: People with a family history of 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 people 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 people with IPF are male.
Age: Most patients with IPF are over the age of 50 years.

Idiopathic pulmonary fibrosis scars and 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.

Occupational and Environmental Factors

Long-term exposure to a number of 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 Treatment

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 the following factors:

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

Medications

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

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

Medical Conditions

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

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

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

Many patients with idiopathic pulmonary fibrosis may also have gastroesophageal reflux disease (GERD), a condition that occurs when acid from the stomach flows back into the 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 and GERD.

Diagnosis of Idiopathic Pulmonary Fibrosis (IPF)

According to the Canadian Lung Association, to diagnose IPF, a doctor will assess any symptoms, take a full medical history, and carry out a physical examination.

During the physical examination, they may check whether a person 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 IPF. These tests may include:

Breathing test: This test checks how well the lungs breathe air in and out and how well they oxygenate the blood.
Chest X-ray: To look for signs of disease and progression.
Blood tests: To identify other reasons for lung scarring.
High resolution chest CT scan: To look for scarring in the lungs.
Lung biopsy: To confirm a diagnosis if this is not possible based on a CT scan.
Pulse oximetry and arterial blood gas tests: This test measures how much oxygen is in your blood.
Spirometry: It measures how well your lungs are working by showing how much air you can blow out.

Complications of Idiopathic Pulmonary Fibrosis (IPF)

Living with IPF may cause anxiety, stress, and depression. A doctor may recommend:

A patient support group
Cognitive behavioral therapy (CBT)
Medications, such as antidepressants

According to a 2018 article in the journal Medical Sciences, 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

Complications of pulmonary fibrosis may include the following:

High blood pressure in your lungs (pulmonary hypertension): Unlike systemic high blood pressure, this condition affects only the arteries in the lungs. It begins when the smallest arteries and capillaries are compressed by scar tissue, causing increased resistance to blood flow in the lungs.
This in turn raises pressure within the pulmonary arteries and the lower right heart chamber (right ventricle). Some forms of pulmonary hypertension are serious illnesses that become progressively worse and are sometimes fatal.
Right-sided heart failure (cor pulmonale): This serious condition occurs when the heart’s lower right chamber (ventricle) has to pump harder than usual to move blood through partially blocked pulmonary arteries.
Respiratory failure: This is often the last stage of chronic lung disease. It occurs when blood oxygen levels fall dangerously low.
Lung cancer. Long-standing pulmonary fibrosis also increases your risk of developing lung cancer.
Lung complications. As pulmonary fibrosis progresses, it may lead to complications such as blood clots in the lungs, a collapsed lung or lung infections.

Treatment of Idiopathic Pulmonary Fibrosis (IPF)

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

The treatment options depend on the stage of IPF but may include the following:

Antacids: These helps treat GERD if patients also have this condition.
Kinase inhibitors: This type of medication can help slow down a loss of lung function and may prevent a sudden worsening of IPF.
Lung transplant: This surgery may be necessary for people with advanced IPF.
Oxygen therapy: Initially, a person may need this therapy after exertion. In the later stages of IPF, they may need it continually.
Ventilator support: A person may need this support if their breathing problems become severe.

Other forms of treatment depend on the person’s symptoms. For example, if a patient has developed a lung infection, they may require 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:

Breathing exercises
Counseling
Education on IPF and how to manage the condition
Nutritional advice
Physical activity to strengthen the body

Bay Biosciences is a global leader in providing researchers with high quality, clinical grade, fully characterized human tissue samples, bio-specimens, and human bio-fluid collections.

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.

Bay Biosciences maintains and manages its own biorepository, the human tissue bank (biobank) consisting of thousands of diseased samples (specimens) and from normal healthy donors available in all formats and types.

Our biobank procures and stores fully consented, deidentified and institutional review boards (IRB) approved human tissue samples and matched controls.

All our human tissue collections, human specimens and human bio-fluids are provided with detailed, samples associated patient’s clinical data.

This critical patient’s clinical data includes information relating to their past and current disease, treatment history, lifestyle choices, biomarkers, and genetic information.

Patient’s data is extremely valuable for researchers and is used to help identify new effective treatments (drug discovery & development) in oncology, and other therapeutic areas and diseases.

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.

Bay Biosciences is a global leader in collecting and providing human tissue samples according to the specified requirements and customized, tailor-made collection protocols.

Please contact us anytime to discuss your special research projects and customized human tissue sample requirements.

Bay Biosciences provides human tissue samples (human specimens) from diseased and normal healthy donors which includes:

Peripheral whole-blood
Amniotic fluid
Bronchoalveolar lavage fluid (BAL)
Sputum
Pleural effusion
Cerebrospinal fluid (CSF)
Serum (sera)
Plasma
Peripheral blood mononuclear cells (PBMC)
Saliva
Buffy coat
Urine
Stool samples
Aqueous humor
Vitreous humor
Kidney stones (renal calculi)
Other bodily fluids from most diseases including cancer.

We can also procure most human bio-specimens, special collections and requests for human samples that are difficult to find. All our human tissue samples are procured through IRB-approved clinical protocols and procedures.

In addition to the standard processing protocols, Bay Biosciences can also provide human plasma, serum, and PBMC bio-fluid samples using custom processing protocols; you buy donor-specific collections in higher volumes and specified sample aliquots from us.

Bay Biosciences also provides human samples from normal healthy donors; volunteers, for controls and clinical research, contact us Now.

日本のお客様は、ベイバイオサイエンスジャパンBay Biosciences Japanまたはhttp://baybiosciences-jp.com/contact/までご連絡ください。