Management Of Idiopathic Pulmonary Fibrosis

 Idiopathic Pulmonary Fibrosis

Pulmonary fibrosis is a chronic, progressive lung disorder characterized by scarring (fibrosis), thickening, hardening, and stiffening of the lung tissues. When the cause of the disease is unknown, it is termed “idiopathic.” Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial lung disease (ILD). It affects the connecting tissue in the lung and the alveoli (air sacs inside the lungs). There are more than 200 types of interstitial lung diseases, which affects the lung’s capacity to absorb oxygen from the air and transport them into the bloodstream effectively. This is due to varying degrees of scarring, inflammation, or both. The disease usually affects people between the ages of 50 and 70, it is rare in people under 50. 

Symptoms Of Idiopathic Pulmonary Fibrosis: At the early stages of the disease, many people with IPF have no symptoms. The symptoms tend to develop gradually and get slowly over time. When the disease progresses, the symptoms can include 

• Shortness of breath when exercising or doing other activities

• Continuous dry, hacking cough

• Loss of weight and appetite

• Tiredness 

• Low-grade fever

• Joint stiffness

• Muscle aches and pains

• Discomfort in chest

• Clubbing of the fingers or toes

Risk Factors Of Idiopathic Pulmonary Fibrosis: Although the word idiopathic means unknown cause, certain risk factors increase the chance of developing idiopathic pulmonary fibrosis. Some of these factors are 

• Family history of pulmonary fibrosis

• Older age and male gender

• environmental factors 

• Cigarette smoking

• Autoimmune diseases

• Gastroesophageal reflux disease (GERD)

• Certain chemotherapy and heart medications

• Lung infections

Idiopathic Pulmonary Fibrosis Diagnosis: When a patient is suspected of idiopathic pulmonary fibrosis, the healthcare provider may advise them to take one or more tests to diagnose the condition.

1. Physical Examination: Your healthcare professional may ask about your medical and medication history, social and work history, family history, and home environment. They may also use a stethoscope to listen to certain crackling sounds from the scarred lungs. 

2. Blood Tests: There are no specific blood tests for IPF, but  certain blood tests provide information about the other comorbid conditions and track the disease progression(how IPF affects your body over time)

3. Pulmonary Function Test: It measures how much air the lungs can hold and how well they work.

4. Chest X-ray and Chest CT scan: To get detailed images of your lungs for detecting the severity of IPF, your doctor may ask to take these tests.   

5. Lung Biopsy: To confirm the presence of idiopathic pulmonary fibrosis biopsy may be required. A small incision is made in the ribs to remove the lung tissue for the test. 

Management Of Idiopathic Pulmonary Fibrosis

There is no permanent cure for IPF, but the treatment can focus on relieving symptoms, decreasing the progression, and improving the quality of life. The treatment plan includes certain oral medications, oxygen therapy, pulmonary rehabilitation, and lung transplant at a severe stage. Other than these, potential self-care preventive measures such as quitting smoking, eating healthy foods, being proactive, preventing yourself from infections, and breathing exercises should be included.  

There are currently two medications approved for treating idiopathic pulmonary fibrosis. One of these medications is Bronchonib 150 mg capsule. It works by inhibiting certain abnormal proteins called tyrosine kinase. Bronchonib 150 mg capsule interferes with certain pathways that involve in the formation of lung fibrosis. The medicine is not recommended in patients with moderate or severe liver injury.