The term ‘pulmonary’ refers to the lung, while the term ‘fibrosis’ refers to scar tissue, which is comparable to scars that grow on the skin as a result of an old injury or surgery. Pulmonary fibrosis (PF) is a type of chronic lung disease that involves the scarring and thickening of the lace-like tissue around the air sacs — known as alveoli — in the lungs.
Over time, these changes can severely disrupt lung function, often with fatal consequences. It can make the lungs less flexible and reduce their ability to expand and contract with each breath, which can make breathing more difficult. Other complications include pulmonary hypertension and respiratory failure, which occur when the lungs are unable to carry enough oxygen into the bloodstream without assistance. This stops the brain and other organs from receiving adequate oxygen.
In this post, we will cover some key information about pulmonary fibrosis.
Symptoms
The course of pulmonary fibrosis — and the severity of symptoms — can vary greatly from person to person. Some people become severely unwell very soon. Others have mild symptoms that worsen gradually over months or years.
Symptoms of pulmonary fibrosis can include:
- Shortness of breath
- Persistent dry cough
- Fatigue and exhaustion
- Loss of appetite
- Rapid weight loss
- Chest discomfort
- Clubbing of the fingers and toes
Causes
Pulmonary fibrosis is a complex disease that can be caused by a multitude of factors. But in most cases, the exact cause of pulmonary fibrosis cannot be pinpointed. Researchers have several theories about what might trigger PF, but it is believed to be related to inflammation in the lungs that triggers the development of scar tissue. This can be caused by a variety of factors, including:
Environmental factors
Regularly breathing in chemicals or certain airborne toxins such as asbestos, silica dust, hard metal dust, and coal dust can damage the lungs.
Medications
Certain medications, including some chemotherapy drugs, antibiotics such as nitrofurantoin, drugs used to treat irregular heartbeats, and anti-inflammatory drugs such as methotrexate can cause pulmonary fibrosis.
Radiation therapy
Radiation therapy for lung or breast cancer can damage the lung tissue and can put you at an increased risk of pulmonary fibrosis.
Genetics
Medical experts do believe that some types of pulmonary fibrosis may be caused by genes that run within families.
Autoimmune diseases
Some medical conditions, such as rheumatoid arthritis and lupus, can cause lung damage that leads to pulmonary fibrosis.
Infections
Certain viral and bacterial infections, such as pneumonia and tuberculosis, can cause pulmonary fibrosis.
Idiopathic
In some cases, the underlying cause of pulmonary fibrosis is unclear. This is called idiopathic pulmonary fibrosis (IPF), which is a type of pulmonary fibrosis that occurs in people who have no known cause for the disease.
Diagnosis
To diagnose pulmonary fibrosis, your doctors may evaluate your medical and family history, discuss your signs and symptoms, review any dust, gas, or chemical exposure you’ve had, and perform a physical exam. He or she may also use imaging tests such as a chest X-ray or CT scan, as well as pulmonary function tests to measure how well your lungs are working. Blood tests may also be ordered by your doctor to check your liver and kidney function, as well as to rule out other illnesses.
Treatment
There is no cure for pulmonary fibrosis, but treatment can help manage symptoms and slow the progression of the disease. This may include medications to reduce the rate at which scarring worsens, immunosuppressants, oxygen therapy, pulmonary rehabilitation, and lung transplant in severe cases.
Prognosis
The outlook for pulmonary fibrosis is highly variable depending on the underlying cause and the severity of the disease. Some people may respond well to treatment, while others may experience a more rapid progression of the disease or find the breathlessness debilitating.
Key Takeaway
Pulmonary fibrosis (PF) is a type of rare lung disease that causes the tissue around the air sacs within the lungs to become thickened. One reason pulmonary fibrosis is frequently misdiagnosed is that its symptoms are similar to those of other lung illnesses. The majority of persons with PF develop symptoms between the ages of 50 and 70 yrs. If you experience any of the symptoms listed above, consult your doctor immediately and be persistent if your lung health does not improve. Also, inform your doctor about any family history of lung disease or previous exposure to risk factors.
