Bay Biosciences provides high quality, clinical grade, biopsy tissue samples, FFPE blocks with matched cryogenically preserved sera (serum) and plasma biofluid samples from patients diagnosed with pulmonary fibrosis.
The K3-EDTA plasma, sera (serum) and PBMC biofluid specimens are processed from unique pulmonary fibrosis patient’s peripheral whole-blood using customized collection and processing protocols.
Pulmonary Fibrosis Overview
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung condition. 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.
Pulmonary fibrosis is a scarring of the lungs. The NHLBI state that 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.
Stages of Pulmonary Fibrosis
According to a 2014 article, 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 Fibrosis
At this stage of IPF, people may show mild symptoms of IPF and receive an initial diagnosis.
In some cases, though, people may not have any symptoms until the moderate stage of IPF.
Moderate Fibrosis
At this stage, there will be a large amount of scarring in the lungs, and people may need oxygen therapy.
Advanced Fibrosis
Symptoms are severe at this stage, and a lung transplant may be necessary.
Different 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:
- Autoimmune Pulmonary Fibrosis: Due to autoimmune conditions, such as rheumatoid arthritis (RA) or Sjögren’s Syndrome.
- Drug-induced Pulmonary Fibrosis: Due to the previous or current use of drugs that affect the lungs, such as chemotherapy, amiodarone (Pacerone), or nitrofurantoin (Macrobid).
- Environmental Pulmonary Fibrosis: Due to exposure from lung irritants, such as mold.
- Occupational Pulmonary Fibrosis: Due to exposure to dust or fumes, such as asbestos or silica.
- Radiation-induced Pulmonary Fibrosis: Due to radiation treatment of the chest.
Idiopathic pulmonary fibrosis (IPF) differs from the other form of pulmonary fibrosis because it has no known cause.
Signs and Symptoms of Pulmonary Fibrosis
According to the NHLBIT, the symptoms of IPF develop gradually over time for some people but appear more quickly for others.
Following are the signs and symptoms of idiopathic pulmonary fibrosis (IPF):
- A dry cough that worsens and can involve bouts of uncontrollable coughing
- Aching muscles and joints
- Clubbing, which is where the tips of the fingers or toes become wider and rounder
- Fatigue
- Feeling short of breath, which tends to occur when exercising but may worsen over time and cause patients to feel out of breath when resting
- General feeling of being unwell
- Loss of appetite
- Pain or tightness in the chest
- Unexplained weight loss over time
Causes of Pulmonary Fibrosis
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.
Other risk factors for IPF include:
- Age: Most people with IPF are over the age of 50 years.
- Being male: Approximately 75% of people with IPF are male patients.
- Genetics: Patients with a family history of IPF have a higher risk of developing idiopathic pulmonary fibrosis (IPF), particularly if a first degree relative, such as a parent or sibling, has IPF. However, IPF can also occur sporadically.
- Smoking: About 75% of patients with idiopathic pulmonary fibrosis (IPF) are smokers or have previously been smokers.
- Gastroesophageal reflux disease (GERD): Roughly 75% of people with IPF have gastroesophageal reflux (GERD) or heartburn symptoms.
Complications of Pulmonary Fibrosis
Living with IPF may cause anxiety, depression and stress. Your health care provider may recommend the following:
- A patient support group
- Cognitive behavioral therapy (CBT)
- Medications, such as antidepressants
According to an article in the journal Medical Sciences, possible complications of idiopathic pulmonary fibrosis (IPF) can include the following:
- Difficulty having a sound sleep
- 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
Diagnosis of Pulmonary Fibrosis
According to 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, your doctor may check whether the patients have:
- Blue hands and feet
- Clubbed toes or fingers
- High pitched crackles coming from the lungs
A combination of tests may help your healthcare provider confirm a diagnosis of idiopathic pulmonary fibrosis (IPF). These tests may include:
- Breathing test: To check how well the lungs breathe air in and out and how well they oxygenate the blood.
- CT scan: To look for scarring in the lungs
- Chest X-ray: Identify for signs of disease and progression.
- Blood tests: To identify other reasons for lung scarring.
- Lung biopsy: Evaluate and confirm a diagnosis if this is not possible based on a CT scan.
Treatment of Pulmonary Fibrosis
According to the NHLBI, although there is currently no cure for idiopathic pulmonary fibrosis (IPF), treatments may help slow the progression of IPF and improve quality of life.
The treatment options depend on the stage of idiopathic pulmonary fibrosis (IPF) a patient has and may include the following:
- Antacids: These helps treat GERD if the IPF patients also have this condition.
- Kinase Inhibitors: These types of medications 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 patients with advanced IPF.
- Oxygen therapy: Initially, a patient with IPF may need this therapy after exertion. In the later stages of IPF, they may need it continually.
- Ventilator support: IPF patients may need this support if their breathing problems become severe.
Other forms of treatment depend on the individual patient’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 the following:
- Breathing exercises
- Counseling
- Education on IPF and how to manage the condition
- Nutritional advice
- Physical activity to strengthen the body
Management of Pulmonary Fibrosis
Keeping to a treatment plan can help patients with idiopathic pulmonary fibrosis (IPF) manage the disease and improve their quality of life.
Steps that patients can take to manage idiopathic pulmonary fibrosis (IPF) include the following:
- Avoiding places or situations that may make breathing more difficult, such as being at high altitudes
- Attending regular checkups
- Avoiding substances that can irritate the lungs, such as chemicals, dust and other pollutants
- Eating to support heart and lung health, such as eating smaller meals to reduce fullness, which may ease breathing
- Joining support groups, having counseling, and talking with family and friends can help people take care of their mental health
- Keeping track of symptoms and letting a healthcare professional know if they worsen
- Maintaining a moderate weight
- Refraining from smoking cigarettes and avoiding secondhand smoke
- Staying physically active and keeping up regular, moderate exercise, such as walking or using an exercise bike
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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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