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What is pulmonary fibrosis (PF)? -

PF is caused by scars in the lungs. It’s essentially a buildup of fibrous connective tissues in the lungs.

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What is PF? -

These scars then restrict the movement of oxygen into the bloodstream, causing breathing difficulties.

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Types and causes of PF -

This form of interstitial lung disease is divided into different types and categories. Let’s look at them, as well as the causes, in the next slides.

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Idiopathic pulmonary fibrosis (IPF) -

This is when the PF has no known cause. It’s actually the most common type of PF.

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PF caused by diseases -

Some autoimmune diseases, such as rheumatoid arthritis and scleroderma, to name a few, can cause PF.

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PF caused by diseases -

Other conditions, such as gastroesophageal reflux disease (GERD), can also injure the lungs and increase the risk of developing PF.

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Familial PF -

It is considered familial PF when two or more members of the same family have IPF or other forms of idiopathic interstitial pneumonia. This is, however, quite rare.

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PF caused by exposure -

Exposure to hazardous materials such as asbestos, silica, coal and grain dust, and even some animal droppings, can lead to PF.

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Radiation -

Exposure to radiation, during cancer treatment for instance, can cause damage to the lungs and cause PF.

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Medications -

Some medications can also contribute to the development of PF. These include some chemotherapy drugs, heart medications, anti-inflammatories, and some antibiotics.

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Risk factors -

Smokers, people with certain occupations (where there is exposure to harmful elements), cancer patients subject to chemo and/or radiotherapy, and genetic factors (family history) are some of the risk factors for PF.

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Risk factors -

PF affects mostly men over the age of 50, but there are many other risk factors, in addition to sex and age, that can contribute to the development of PF.

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Symptoms -

PF blocks oxygen from entering the bloodstream, so the main symptom is indeed shortness of breath, particularly during exercise. But there’s more.

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Other symptoms -

Other PF symptoms can include tiredness, a dry cough, aching muscles and joints, weight loss, and clubbing (widening) of the fingers and toes.

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Diagnosis -

PF is not easy to diagnose because its symptoms are very similar to other lung diseases. A mix of imaging, lung function, and sometimes even surgical tests are performed.

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Physical exams -

Your doctor should go through your health history and perform a physical exam (listen to your lungs). A six-minute walk test might also be performed.

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Imaging tests -

Imaging tests are usually done. While a standard chest x-ray might not show PF, there are other exams, such as an HRCT scan, that can.

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Echocardiograms -

An echocardiogram can also be done to measure how much pressure is occurring on the right side of your heart.

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Lung function tests -

Your doctor will probably also make use of lung function tests, such as blowing into a spirometer ( a device that measures the volume of air inspired and expired).

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Other tests -

Pulse oximetry is usually also used to measure the level of oxygen in your blood. Other tests such as an exercise stress test, arterial blood gas test (to measure oxygen and carbon dioxide levels), and blood work might also be prescribed.

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Further exams -

A bronchoscopy might also be done to see the inside of your airway. Other exams might be prescribed to rule out other conditions.

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Life expectancy and complications -

While developments in medicine have allowed PF patients to have a better quality of life and live longer, complications may arise.

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Pulmonary hypertension -

Scar tissue can potentially compress arteries and capillaries, which can lead to high blood pressure in the lungs.

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Right-sided heart failure (cor pulmonale) -

This is caused by high blood pressure in the arteries of the lung and right ventricle of the heart, putting a huge strain on it.

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Respiratory failure -

This happens in extreme cases, when oxygen levels fall drastically to low levels.

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Cancer and other lung diseases -

PF can also increase the risk of developing lung cancer, as well as blood clots, a collapsed lung, and lung infections.

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Treatment -

Managing PF involves a number of strategies, depending on how serious it is. This includes drugs to slow down the progression of the disease.

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Treatment -

Because many people with PF also suffer from gastroesophageal reflux disease, anti-acid medications are usually prescribed.

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Treatment -

Oxygen therapy is also used in the treatment of PF. Lifestyle elements such as diet and exercise also play a major role in the treatment. The last resort in the treatment of PF is a lung transplant.

Sources: (Mayo Clinic) (American Lung Association)

See also: What happens when you stop smoking

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