Bay Biosciences provides high quality, clinical grade fresh frozen lung biopsy tissue with matched bronchoalveolar lavage fluid (BAL), sera (serum) and plasma biofluid samples from unique patients diagnosed with Idiopathic Pulmonary Fibrosis (IPF).
Detailed clinical data and pathology annotations including patient’s corresponding diagnostics, high-resolution computed tomography (HRCT) test values associated with the lung biopsy tissue and matched BAL, sera (serum) and plasma biofluid samples are provided for research, development, diagnostics and discovery.
Idiopathic Pulmonary Fibrosis (IPF) Overview
Idiopathic pulmonary fibrosis (IPF) is a lung disease that results in scarring (fibrosis) of the lungs for an unknown reason. Over time, the scarring gets worse and it becomes hard to take a deep breath and the lungs cannot take in enough oxygen. Idiopathic pulmonary fibrosis (IPF) scar tissue is thick, like the scars you get on your skin after a cut. It slows oxygen flow from the lungs to the blood, which can keep the body from working normally as it should.
Idiopathic pulmonary fibrosis (IPF) is a form of interstitial lung disease, primarily involving the interstitium (the tissue and space around the air sacs of the lungs), and not directly affecting the airways or blood vessels. There are many other kinds of interstitial lung disease that can also cause inflammation and/or fibrosis. There’s no cure for idiopathic pulmonary fibrosis (IPF). For most idiopathic pulmonary fibrosis (IPF) patients, symptoms don’t get better, but treatments can slow the damage to the lungs.
The cause of idiopathic pulmonary fibrosis (IPF) is not completely understood. No one knows what causes idiopathic pulmonary fibrosis or why some people get it. “Idiopathic” means of unknown cause. Common risk factors for Idiopathic pulmonary fibrosis (IPF) include genetics (family history), cigarette smoking, acid reflux, heartburn. Idiopathic pulmonary fibrosis (IPF) disease usually affects male patients over the age of 50 years.
There is no cure for idiopathic pulmonary fibrosis (IPF) and there are currently no medical procedures or medications that can remove the scarring from the lungs. Treatments are used to slow progression of the lung scarring, and may not necessarily lessen the symptoms of cough and shortness of breath or breathlessness.
Causes of Idiopathic Pulmonary Fibrosis (IPF)
Causes of developing idiopathic pulmonary fibrosis (IPF) disease are unknown. “Idiopathic” means of unknown cause. Following are the common risk factors for idiopathic pulmonary fibrosis (IPF):
- Genetics (family history): Up to 20% of IPF patients have another family member with an interstitial lung disease. If more than one member of your family has IPF, the disease is called familial pulmonary fibrosis.
- Cigarette smoking: Approximately 75% of people with IPF are current or previous cigarette smoking history.
- Acid reflux -gastroesophageal reflux disease (GERD): Approximately 75% of patients with IPF have symptoms of acid reflux and heartburn.
- Male sex: Approximately 75% of patients with IPF are male.
- Age: Almost all patients with IPF are over the age of 50 years
Idiopathic pulmonary fibrosis (IPF) 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 toxins include:
- Silica dust
- Asbestos fibers
- Hard metal dusts
- Coal dust
- Grain dust
- Bird and animal droppings
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 disease
Medications
Many drugs can damage your lungs, especially medications such as:
- 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 (arrhythmias), 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.
- 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 a number of the following conditions:
- Dermatomyositis
- Polymyositis
- Mixed connective tissue disease
- Systemic lupus erythematosus
- Rheumatoid arthritis
- Sarcoidosis
- Scleroderma
- Pneumonia
Many substances and conditions can lead to pulmonary fibrosis. Even so, in most cases, the cause is never found. Researchers have several theories about what might trigger idiopathic pulmonary fibrosis (IPF), including viruses and exposure to tobacco smoking. Also, some forms of idiopathic pulmonary fibrosis run in families, and heredity may play a role in idiopathic pulmonary fibrosis.
Many people with idiopathic pulmonary fibrosis may also have gastroesophageal reflux disease (GERD), 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 and GERD.
Signs and Symptoms of Idiopathic Pulmonary Fibrosis (IPF)
The course of idiopathic pulmonary fibrosis (IPF) and the severity of the symptoms vary considerably from person to person. Some patients become sick very quickly with severe symptoms. While others have moderate symptoms that worsen more slowly, over months or years. Some 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 several weeks.
Following are the two main symptoms of idiopathic pulmonary fibrosis (IPF):
The two main symptoms of IPF are:
- Shortness of breath: (dyspnea): Usually the shortness of breath of IPF first appears during exercise. Shortness of breath can affect day-to-day activities such as showering, climbing stairs, getting dressed and eating. As scarring in the lungs gets worse, shortness of breath may prevent all activities.
- Chronic cough: About 85% of people with IPF have a chronic cough that has lasts longer than 8 weeks. This is often a dry cough, but some people may also cough up sputum or phlegm.
Other symptoms of idiopathic pulmonary fibrosis (IPF) may include:
- Aching muscles and joints
- Chest pain or tightness
- Unexplained weight loss
- Loss of appetite
- Fatigue
- Change of finger shape, widening and rounding of the tips of the fingers or toes “clubbing“.
The course of pulmonary fibrosis, and the severity of symptoms can vary considerably from person to person. Some patients become ill very quickly with severe symptoms. Others have moderate symptoms that worsen more slowly, over months or years. Some 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. Patients who have acute exacerbations may be placed on a mechanical ventilator. Antibiotics, corticosteroid medications or other medications may be prescribed to treat an acute exacerbation.
Risk Factors of Idiopathic Pulmonary Fibrosis (IPF)
Following are the risk factors that make you more susceptible to idiopathic pulmonary fibrosis (IPF):
- Age: Although idiopathic pulmonary fibrosis has been diagnosed in children and infants, the disorder is much more likely to affect middle-aged and older adults.
- Sex: Idiopathic pulmonary fibrosis is more likely to affect men than women.
- Smoking: Smokers and former smokers develop idiopathic pulmonary fibrosis (IPF) than do people who have never smoked. Idiopathic pulmonary fibrosis (IPF) can occur in patients with emphysema.
- Certain occupations. You have an increased risk of developing idiopathic pulmonary fibrosis (IPF) if you work in mining, farming or construction or if you’re exposed to pollutants known to damage your lungs.
- Cancer treatments. Having radiation treatments to your chest or using certain chemotherapy drugs can increase your risk of pulmonary fibrosis.
- Genetic factors. Some types of pulmonary fibrosis run in families, and genetic factors may be a component.
Complications of Idiopathic Pulmonary Fibrosis (IPF)
Complications of idiopathic pulmonary fibrosis (IPF) may include:
- 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 idiopathic pulmonary fibrosis also increases the risk of developing lung cancer.
- Lung complications. As idiopathic pulmonary fibrosis progresses, it may lead to complications such as blood clots in the lungs, a collapsed lung or lung infections.
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