Idiopathic pulmonary fibrosis is a kind of interstitial disease of the lung or ILD which have five broad categories. Described as scarring or fibrosis of the lungs, idiopathic; in fact, means “of unknown cause.”

Interstitial lung diseases are an umbrella term of lung diseases which comprise an excess of 200 disorders that are characterized by scar/fibrosis or inflammation of the lungs. The main cause for ILD remains elusive despite intensive research. Idiopathic pulmonary fibrosis is one form of ILD due to an unknown cause. It is a degenerative condition that reduces the quality of life and studies show that the survival rate among people with IPF is around 3-5 years from the time they are diagnosed. The study indicates that 50 percent of those who had idiopathic pulmonary fibrosis have died and only 50 percent are alive.

Idiopathic pulmonary fibrosis causes problems like shortness of breath, dry hacking cough, tiredness, muscle ache, joint pain, unintended weight loss, widening of the fingertips and toes. The symptoms gradually worsen over time and it is known as acute exacerbation of idiopathic pulmonary fibrosis. The immune system is the body’s defense system, it keeps the body healthy and helps fight infections and regulates the response to prevent autoimmune disease As idiopathic pulmonary fibrosis is caused by genetic defects and it impacts how the immune system works.
The lack of a normal immune system leads to serious medical problems related to the respiratory, neurological and digestive systems. These can also lead to malfunctioning of the body parts, ultimately resulting in organ failure. Idiopathic pulmonary fibrosis also increases the risk of cancer, which is why it is essential to keep track of the growth of the disease, and its effects on the body.

Diagnosing idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis is diagnosed by evaluating the symptoms and making sure it isn’t being confused with another lung disease. The doctor conducts a lot of tests including;

• Complete medical history
• Complete physical examination
• Chest imaging
• Chest x-ray
• High resolution computed tomography (HRCT) scan
• Pulmonary function tests
• Arterial blood gas
• Exercise test
• Assessment of blood oxygen levels during exertion
• Blood tests
• Bronchoscopy with bronchoalveolar lavage
• Surgical lung biopsy

Your doctor may order tests which are based on your medical history and physical examination. Your healthcare provider could also ask you to see a specialist – a pulmonologist (lung specialist) to confirm the diagnosis and treat the condition.

Managing idiopathic pulmonary fibrosis
You need to adopt a proper lifestyle which includes healthy eating, regular exercise, rest, and support from others. You need to take care of your emotional and mental health, give up smoking, avoid infections, take regular medication, oxygen therapy, use breathing techniques, and determine whether you need lung transplantation.

There are four stages to idiopathic pulmonary fibrosisIPF recently diagnosed— the stage when you need oxygen but not when at rest, needing oxygen 24 hours a day, needing advanced oxygen, needing high flow oxygen.