Health explainer: What is idiopathic pulmonary fibrosis and its effects?

Renowned musician Zakir Hussain who weaved magic on tabla passed away on Monday morning after being admitted in a San Francisco hospital due to deteriorating health condition As per the family statement lsquoidiopathic pulmonary fibrosisrsquo is stated as the cause of deathPulmonary fibrosis or idiopathic pulmonary fibrosis is a chronic progressive lung disease that can severely affect the respiratory functions of the body Middaycom speaks to Dr Kamlesh Pandey Consultant Chest Physician Wockhardt Hospitals Mira Road and Dr Sheetal Chaurasia Consultant ndash Pulmonary Medicine Manipal Hospital Whitefield who explain the symptoms and causes of the disease also stressing on why early diagnosis is essential to slow down its progressionWhat is pulmonary fibrosisPandey Pulmonary fibrosis is a term used when there is a progression of fibrosis in the lungs It is a diffuse lung disease Lung is an elastic organ During pulmonary fibrosis the lung function declines over a period of time ie elasticity of the lungs goes down Over time the oxygen levels also go down causing difficulty in breathingChaurasia Pulmonary fibrosis is scarring of the lungs due to some injury It is an umbrella term that covers multiple diseases that by different mechanisms cause lung injury and initiate a cellular and inflammatory reaction ndash the end result of which is permanent scarring of the lung known as fibrosisPulmonary fibrosis causes stiffening of the lungs by replacing normal airway cells with scar tissues This hampers the gas exchange as well as decreases the compliance of the lungs In simple terms the lungs become thick stiff and shrunken and patients will have to put in more effort to breathe normallyWhat are the causes of this respiratory condition Are there any risk factorsPandey There could be multiple causes falling into categories like autoimmune connective tissue diseases chronic hypersensitivity pneumonitis certain infections like COVID19 etc Smoking is a major risk factorChaurasia There are over 200 causes of pulmonary fibrosis with the most common ones being environmental druginduced toxinrelated autoimmune diseases occupational and idiopathicIrrespective of the cause of pulmonary fibrosis the majority of these conditions are progressive in nature and the speed of progression is variable and unpredictableWhat is idiopathic pulmonary fibrosis Pandey A lot of times no particular cause is found for the disease This is called idiopathic pulmonary fibrosis and is more prevalent from what I have observed in my practiceChaurasia In a great number of cases the cause of pulmonary fibrosis couldnrsquot be ascertained and these are called idiopathicWhat are some symptoms one should look out for in order to ensure early diagnosisPandey Some common symptoms include persistent cough breathlessness that is progressive over a period of time frequent infections during seasonal changes that exacerbate the condition etcChaurasia Clinical features are nonspecific with the majority of patients presenting with chronic dry cough in the early stage and slowly progressive breathlessness at a more advanced stage The diagnosis may be difficult at the early stage because symptoms are often nonspecific and chest xray may not identify the disease at the early stage Thus in the early stage it is often misdiagnosed as allergic bronchitis or asthmaIs there a treatment for the conditionPandey Pulmonary fibrosis is irreversible Therersquos no cure The treatment offered is only to delay the progression of the diseaseChaurasia The main goal of the treatment is preventing or slowing the progression of the disease Pulmonary fibrosis is an end outcome of a variety of insults to the lung and is irreversible hence early diagnosis is paramountOnce the disease is significantly progressed it can cause respiratory failure and subsequently right heart failure The only treatment available at this stage is a lung transplant or heartlung transplant depending on the severity However if diagnosed earlier the progression of the disease could be slowed down or checked by certain antiinflammatory and antifibrotic medications So a chronic cough and breathlessness not responding to regular treatment should be evaluated at an early stage by a pulmonologistCan pulmonary fibrosis be prevented Pandey There is no particular way to prevent it But if the cause is identified the exposure to those elements can be avoided Smoking should be avoided If someone in the family has suffered from pulmonary fibrosis people should be more careful and go for active screeningsWhat are some things that pulmonary fibrosis patients should keep in mindPandey Patients should be vaccinated against common infections like flu and pneumonia Certain exercises and rehabilitation practices as advised by doctors can aid in preserving lung function and improving quality of lifeDisclaimer This is for informational purposes only and does not replace professional medical advice Kindly consult a qualified medical expert for personalised guidanceAlso ReadLung cancer rising among nonsmokers in India doctors raise concern