Pulmonary fibrosis

Pulmonary fibrosis is the scarring of the deep tissues of the lungs which can develop either slowly or quickly. The tissue gets thick and stiff making it difficult to breathe, reducing the oxygen levels in the blood.

Symptoms

The most common symptoms of pulmonary fibrosis are:

Shortness of breath especially while exercising which can become evident even at rest with time
A dry and hacking cough which can become uncontrolled over a period

The other pulmonary fibrosis symptoms which develop with time include:

Fast and shallow breathing
Slow, unintended weight loss
Fatigue (extreme tiredness)
Malaise (feeling unwell)
Painful joints and muscles
Clubbing of tips of fingers and toes

Causes

In most of the cases, pulmonary fibrosis does not have any specific cause. This is called as idiopathic pulmonary fibrosis. The damage can be due to different reasons including:

Cigarette smoking
Certain viral infections
Environmental pollutants such as hard metal dust, silica, animal proteins, bacteria, gasses, and fumes
Gastrointestinal reflux disorder in which the backward movement of acid from the stomach into the lungs can lead to pulmonary fibrosis
Medical conditions including tuberculosis, pneumonia, rheumatoid arthritis, scleroderma, etc.

Risk factors

The below factors can make you more prone to getting pulmonary fibrosis

Genetics - In some families, at least two people have pulmonary fibrosis
Certain medicines such as some antibiotics, heart medicines, and chemotherapeutic drugs
Radiation therapies

Complications

If pulmonary fibrosis is not addressed over time it can result in the below complications:

Pulmonary hypertension: A serious illness in which the blood pressure in the lungs increases. It progressively worsens and can even prove fatal.
Cor pulmonale :This is a condition in which the lower right side of the heart fails to work properly. It can occur due to blockage of the pulmonary arteries.
Lung cancer: The risk of you developing lung cancer increases as the pulmonary fibrosis gets advanced and is left untreated for a long time.
Respiratory failure : This is the final stage of chronic pulmonary fibrosis.

Lung transplant

If no other treatment options are helpful, then a lung transplant is recommended. This is recommended for patients who are in a good physical condition, have no other life-threatening conditions, and are ready to accept the risks of surgery.

Diagnosis

Your doctor takes your family history, medical history and will cross check with your work environment. A physical examination is then performed following other diagnostic tests. They may include one or more of the below:

Test type	Purpose of the test
Chest X-ray	Creates pictures of the structures in the chest Scarring in the lungs can be detected
Breathing test (Spirometry)	To check the breathing ability of the lungs and detect the extent of lung damage
Blood tests	To detect the oxygen levels and signs of infection
CT (Computerized Tomography) scan	Provides sharper and more detailed pictures of the lungs Detects the extent of lung damage
Lung biopsy	Examines the lung tissue under microscope It is the best way to diagnose pulmonary fibrosis. Shows the extent of lung damage
Exercise test	To check the purification of the blood by the lungs

Treatment

Pulmonary fibrosis cannot be cured. However, treatments currently available can prevent further scarring of the lungs and relieve the symptoms so that you can stay active.

The following pulmonary fibrosis treatment options are available:

Medicines : Several medications are available for treating pulmonary fibrosis. The choice of the drug depends on your overall health.

Corticosteroids: These drugs are prescribed to most of the patients to reduce inflammation.
Immune suppressants: Immune suppressant drugs such as cyclosporine and methotrexate may be given to some people in combination with cyclosporines.
Removing the lymph nodes, if cancer has spread to the lymph nodes of the neck
Acetylcysteine and prednisone: This combination can slow down the disease progression in some people.

Oxygen therapy : To help patients with shortness of breath, oxygen supplementation is provided. It is given when the oxygen levels in the blood are low.
Pulmonary rehabilitation :This program involves teaching about the lung exercises, lung diseases, management, support, and counseling.

Self-management

Certain lifestyle changes and home remedies can help the patients with pulmonary fibrosis. They are:

Do not smoke : The first important measure that a person with lung disease should take is to stop smoking. You may take help from a doctor or a counselor for this. Do not allow others to smoke or better avoid such places because second-hand smoking is also injurious.
Eat well :As it becomes difficult to breathe while eating, people with lung diseases may eat less food than others. But to maintain a healthy weight, it is essential to eat adequately.
Get vaccinated :In patients with pulmonary fibrosis, infections of the respiratory system can exaggerate the situation. Therefore, it is advisable for such patients to get flu shots and vaccines against pneumonia.