Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis, a relatively rare name a few years back, has seen a significant jump in cases in the past few years, mainly the ones following COVID-19. Recently, this illness has found its way into the news following being the cause that claimed the life of Zakir Hussain, an eminent musical maestro who redefined the musical landscape for decades with his talent. Let’s learn more about this illness.

What is idiopathic pulmonary fibrosis?

Idiopathic pulmonary fibrosis is one of the more severe lung diseases, which causes scarring and decreases the ballooning of the air sacs, thus leading to low oxygen levels in the body and progressive breathlessness as the scarring increases with age. It is seen to affect people of any age group with varying degrees with varying degrees of progression. Fibrosis causes stiffening of the alveoli and, therefore, also their reduced expansion. Pulmonary fibrosis is a disease that is best caught early so that it can be managed well.

What are the causes of pulmonary fibrosis?

There can be several causes of pulmonary fibrosis, such as follows:

1. Smoking increases the risk of pulmonary fibrosis.
2. Radiotherapy or radiation exposure for a long time
3. Use of medications like amiodarone and bleomycin
4. Working in industries with small particle pollutants in the air, such as silica dust, mold, animal droppings, and asbestos.
5. Lung diseases underlying these include cystic fibrosis, bronchiectasis, mixed connective tissue diseases, scleroderma, and even pneumonia.
6. Idiopathic pulmonary fibrosis can also arise in the absence of any of these kinds of exposures. Sometimes, an increase is also linked to Gastroesophageal reflux.

What are the risk factors for  idiopathic pulmonary fibrosis?

The risk factors for pulmonary fibrosis are as follows:

• Smoking is a risk factor for all kinds of lung diseases, including pulmonary fibrosis
• Working in specific industries like mining or farming can lead to an increased incidence of pulmonary fibrosis.
• People who undergo cancer treatment can also lead to fibrosis, especially if at a young age.
• Genetics alone are responsible for starting the disease process in some people.

What are the symptoms of idiopathic pulmonary fibrosis?

Several symptoms of pulmonary fibrosis can be seen in individuals. Some of the commonly observed  symptoms are as follows:

1. Shortness of breath
2. Extreme fatigue and lack of energy
3. Prolonged loss of weight
4. Persistent dry cough
5. Clubbing of fingers and toes

These symptoms may sometimes get aggravated, leading to an acute exacerbation of the illness, which needs to be dealt with immediately and warrants a visit to the hospital.

What are the methods by which the illness can be diagnosed?

Pulmonary fibrosis can be diagnosed with the help of the following ways, take a medical and physical history of the patient highlighting any signs and symptoms of the illness or of any breathing issues:

• Chest X-ray can help delineate some part of the lung outline that corresponds with the pulmonary fibrosis, such as signs of scarring.
• High-resolution CT scan that can give a more detailed lung image.
• Pulmonary function tests are used to check for the ability of the lung to expand and also to see how well gases like oxygen and carbon dioxide are exchanged. Reduced ability of the lung to take in and absorb oxygen can indicate lung fibrosis
• Sometimes, lung tissue may be taken in a biopsy and examined for any signs of biopsy.
• A bronchoscopy procedure may be done to look for any diseased airways
• A six-minute walk test may be done to check for maintenance of oxygen levels and lung function during exercise. The SPO2 levels are recorded at the start of the test and then after 6 minutes to check the oxygen saturation. A drop in saturation can indicate a lung issue.
• A 2 D Echo may sometimes lead to delineation of the shape of the heart following pulmonary dysfunction.

Multiple methods can be combined to get a complete idea of the extent and severity of lung fibrosis.

What are the treatment methodologies available for lung fibrosis?

There is no permanent method for treatment for Lung fibrosis. Some of the methods that can be tried are as follows:

• Antibiotics, such as pirfenidone and nintedanib, help slow the rate of idiopathic pulmonary fibrosis.
• Corticosteroid: it is used to subdue any underlying inflammation
• Immunosuppressants such as cyclophosphamide to control cells that contribute to lung fibrosis.
• Oxygen supplementation through O2 inhalation is used to maintain oxygen saturation in blood.
• Pulmonary rehabilitation with the help of breathing techniques, chest physiotherapy, and programmed exercise.
• In some cases, a lung transplant may be done to improve life quality and expectancy.
• Palliative care or end-of-life care for severely affected individuals is done to maintain quality of life.
• Implement lifestyle modifications such as stopping smoking, avoiding places where the air is polluted with small particles, eating proper meals, and doing a lot of movement.

What are the symptoms of the worsening of pulmonary fibrosis?

Watch out for the following symptoms for worsening of a case of pulmonary lung fibrosis:

1. Fall in oxygen saturation levels
2. Visible distress during breathing
3. Increased oxygen demand
4. Need for ventilation
5. Any lung infection

In case of an exacerbation, speak to your medical care provider and get a review consultation as soon as possible. Admission is to be done as per the patient’s requirement.

Is there any way to prevent pulmonary fibrosis?

Prevention of pulmonary fibrosis can be done only by avoiding risk factors like smoke, pollution, working in specific industrial environments like that of cotton and asbestos, and also getting the proper medications if you already have a predisposition to lung fibrosis if you have been treated for cancer or have a positive family history. Maintaining a generally healthy lifestyle can also help with this and prolong life spent in good health. Regular checkups in case of discomfort in breathing and other breathing issues are advised to ensure one is on top of their health.

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