Idiopathic Pulmonary Fibrosis
Source: reference.medscape.com

What is Idiopathic Pulmonary Fibrosis(IPF)

Idiopathic Pulmonary Fibrosis (IPF) is a rare lung disease, involving scarring and inflammation of lung tissue. It is a chronic, progressive lung disease.

Idiopathic means unknown cause or mechanism; pulmonary means lung-related; fibrosis means thickening or scarring.

The exact way to define this disease would be: it is a rare, chronic lung disease, caused due to scar tissue build-up in the lungs causing inflammation, stiffening, which affects the sacs in the lungs.

What is scar tissue?

It is a collection of cells and collagen that grow on a site of injury or damage caused regularly. Their growth is regulated by the kind of damage caused.

Symptoms of IPF

Common symptoms of IPF are:

  • Shortness of breath, breathlessness.
  • Dry coughing.
  • Chest pain.
  • Tightness in the chest.
  • Leg swelling.
  • Loss of appetite, gradual weight loss.
  • Clubbing of digits- disfiguring of toe and fingernails.
  • Feeling very tired after doing regular activities.

What is the clubbing of digits?

The finger and toe end become swollen and blue due to lack of oxygen. This is called clubbing of fingers. This also causes disfigured fingers.

Causes of Idiopathic Pulmonary Fibrosis

There are no specifically identified causes but the following could be the underlying reasons:

  • Abnormal growth of tissue in the lung while lung repair; also called as Abnormal Repair Response.
  • Genetic factors.
  • Environmental factors like working in places exposed to sawdust, metal dust, coal dust, stone dust, hay, etc., or related occupations.
  • Cigarette smoking.
  • Acid reflux.
  • In rare cases, viral diseases might also lead to IPF.

How does IPF affect the lungs and body?

In IPF we see abnormal growth of scar tissue, this causes thickening, stiffening of the lung tissue. Normally lung tissues are very flexible and soft allowing easy gas exchange. This scarring increases with time causing damage to lung function.

The tissue around the air sacs becomes thick making it very difficult for oxygen to be absorbed as it can’t fill into the space of the sac. This in turn causes a reduced supply of oxygen to the body, breathlessness, dry cough. As the heart and body cells do not get enough oxygenated blood through the lungs, one feels fatigued doing short tasks, weakness, clubbing of fingers, toes.

This could also cause complications like pneumonia, heart failure, pulmonary hypertension.

Various stages of IPF

The various stages can be mild, moderate, severe based on the pulmonary function test. The initial stage can be termed as mild, here the pulmonary function is most compared to other stages. Severe cases are usually considered as the last stages where the pulmonary function is almost seen to be lost.

What is the diagnosis for IPF?

Diagnosis can be done by various clinical methods:

  • Radiology- chest x-ray can be done, it shows lumps as dark patches on the lung tissue.
  • Pulmonary Function Test- spirometry shows the decrease in the level of vital capacity by a reduced flow of air into and out of the lungs.

Treatment for IPF

Various treatments for IPF are namely:

  • Oxygen therapy- If the patient suffering has low levels of oxygen then artificially supplementary oxygen can be provided with help of oxygen cylinders.
  • Medications- Various medicines used are Pirfenidone(it reduces the risk of death). N-acetylcysteine and triple therapy Nintedanib.
  • Lung transplant- In cases where the lung gets completely damaged, the transplant can be done on finding the eligible donor. This is usually considered as the last resort.
  • Pulmonary rehabilitation- These programs include exercise training, breathing exercises, occupational therapy, psychological counseling. These usually help patients in the early stages of IPF.

Ques. How long can a patient with IPF live?
Ans. The life expectancy of patients is usually 2-5 years after being affected by this disease. In very severe cases they also live less than three years.

Ques. Is IPF a very common disease affecting the population?
Ans.
The frequency of IPF occurring in a population is 13 to 20 people per 100000 people.

Ques. Are clinical trials going on regarding IPF?
Ans.
Yes, clinical trials for idiopathic pulmonary fibrosis are going on in various countries. One can always enroll themselves in these trials in extreme cases. The trials focus on increasing life expectancy by slowing the progress of scaring. Their long-term goal is to find a cure for the disease.

Ques. Can IPF have any related disorders?
Ans.
Numerous other lung disorders can be related to IPF, namely asthma, sarcoidosis, bronchiectasis, chronic obstructive pulmonary disease, drug-induced pulmonary toxicity, lung cancer, pulmonary eosinophilia, etc.

Ques. Is IPF a curable disease?
Ans.
Till now it is not a curable disease, but the symptom progression can be slowed down with the help of various treatments namely oxygen therapy, medication, and lung transplant.

Ques. Is IPF a progressive lung disorder?
Ans.
Idiopathic Pulmonary Fibrosis is a progressive lung disorder because as the abnormal scarring increases the lung function decreases. This causes increased difficulty in respiration.

Ques. What causes the death of IPF patients?
Ans. 
IPF also causes related disorders which usually lead to death, namely heart attack, lung cancer, acute coronary symptoms, excess exertion.

Ques. Does Idiopathic Pulmonary Fibrosis affect a certain age group?
Ans.
IPF is usually seen in people over the age of 50.

Ques. Can IPF be stopped?
Ans.
There is no such method to stop IPF, but one can always take necessary precautions and get medical tests, medications regularly to slow its progression.

Millions of people are being affected by this disease globally. More males than females, in the age group of 60-70. It is important to look out for the early symptoms and get medical attention as soon as possible. Ignorance in cases like these could cost a life.

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