Idiopathic pulmonary fibrosis

Introduction 

Idiopathic pulmonary fibrosis comes under interstitial lung diseases or ILD; these diseases cause scarring or inflammation in the lung.  

Idiopathic pulmonary fibrosis is a persistent lung disease in which scar tissue or fibrosis builds up in your lungs and makes the lungs powerless to transport oxygen into the bloodstream. 

What is pulmonary fibrosis? 

From a technical standpoint, fibrosis equals thickening or scarring of the tissue. Under these circumstances, the lacy walls of air sacs, which are usually thin, are not light anymore. They become thick, stiff, scarred or fibrotic. 

With this scarring, the framework of the lung makes it stiffer and is less effective at delivering oxygen into the bloodstream. Additionally, the fibrosis of the tissue makes it more challenging to expand the lungs. 

It takes additional effort to breathe, and this extra requirement of energy results in shortness of breath. 

Pulmonary fibrosis can be hereditary, too. There are numerous cases of pulmonary fibrosis in which a cause cannot be determined; hence, it is called “idiopathic” pulmonary fibrosis. 

Causes of Idiopathic Pulmonary Fibrosis 

Idiopathic means unknown. The cause of this condition has not yet been discovered. 

Genetic Factors 

After tissue damage, an abnormal tissue repairing process happens, and this expands lung fibrosis. This abnormal repair response results from a combination of genetic and environmental factors. 

Genetic changes are considered risk factors for idiopathic pulmonary fibrosis but account for only a tiny proportion of cases. 

TERT and TERC are mutations in genes that cover 15% of cases in 

familial pulmonary fibrosis and a small proportion of sporadic idiopathic pulmonary fibrosis. 

Telomeres are enzymes that maintain the end structures of the chromosomes. TERC and TERT genes give instructions for making the telomeres. 

Telomerase is built by telomeres. The decline in telomerase function results in atypical shortened telomeres. These shortened telomeres can cause lung cells to cease dividing or to die prematurely. 

Environmental Factors 

Environmental risk factors linked with idiopathic pulmonary fibrosis are: 

• Cigarette smoking 
• Susceptible to dust 
• Viral infections 
• Side effects of a few medications like amiodarone, bleomycin, nitrofurantoin, etc. 

Age Factor 

Idiopathic pulmonary fibrosis occurs in people over 50 years of age, and in this context, age has emerged as a solid risk factor. 

Symptoms 

All the symptoms for this condition are not broad and non-specific; hence, not everyone with these symptoms has idiopathic pulmonary fibrosis. 

• A persistent dry cough 
• Shortness of breath 
• Chest pain or tightness 
• Leg swelling 
• Gradual weight loss 
• Loss of appetite 
• Widened & rounded tips of the fingers and toes 

Treatments 

At present, there is no cure for idiopathic pulmonary fibrosis. However, few treatments can minimise the damage done to the lung. 

• Quit smoking – Cigarettes and tobacco products can damage your lungs. They aggravate your breathing problems. Your doctor can recommend programs to quit smoking. 
• Oxygen therapy – It is a treatment that supplies you with extra oxygen to breathe in. It makes breathing smooth and prevents low blood oxygen levels.  
• Medications – Certain medications may help moderate the progression of idiopathic pulmonary fibrosis. Based on the primary cause and severity of your IPF, you may be prescribed medication therapy to help alleviate symptoms and reduce the progression of your IPF. 
•  Pulmonary rehabilitation – It is a standard treatment used along with medication therapy for those affected by chronic lung disease. It includes Breathing exercises, psychological counselling, nutritional counselling, etc. 
•  Lung Transplant – It is a surgical procedure to change a diseased or failing lung with a healthy lung. It can improve your quality of life and permit you to live longer, but lung donors are rare. 

Tests to Diagnose Idiopathic Pulmonary Fibrosis 

Pulmonary fibrosis can be challenging to diagnose because the symptoms of IPF share similar symptoms with other lung diseases. 

High-resolution CT 

High-resolution CT technology uses a narrow X-ray beam and advanced computer analysis to produce vivid pictures of your body, letting physicians see minute details that would not be possible with a traditional CT. 

Chest X-ray 

A chest X-ray is an imaging test that utilizes X-rays to examine the structures and organs in your chest. It may help your doctor see how your lungs and heart function healthily. 

Biopsy 

A biopsy is a procedure to eliminate cells, tissue or fluid as a sample for examination by a medical pathologist. It is done to diagnose or monitor medical conditions or to plan treatment. 

Other tests 

Pulse oximetry, 

Conclusion 

Unfortunately, Idiopathic pulmonary fibrosis is a progressive and irreversible disease. But with early and specific diagnosis and correct treatments, you can mitigate the progression of the disease. 

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