Since Zakir Hussain, the tabla player and five-time Grammy Award winner, died Sunday in San Francisco of idiopathic pulmonary fibrosis (IPF), there are concerns about how it happens and what age group is at risk for it.
According to Dr Deepak Bhasin, Senior Director, Pulmonology, Critical Care, Max Hospital, Mohali, “It is a chronic, progressive lung disease characterized by scarring (fibrosis) of lung tissue, leading to irreversible loss of lung function. The exact cause of IPF is unknown. This is why it is called idiopathic Unlike lung conditions, IPF specifically targets the interstitium, the tissue surrounding the air sacs (alveoli), which makes it difficult for oxygen to move in the bloodstream. Over time this leads to persistent shortness of breath, fatigue and reduced quality of life,” adds Dr Mahaveer Modi, pulmonologist and sleep specialist at Ruby Hall Clinic, Pune.
Why do scars occur in IPF?
The exact cause of the initial injury to the lung tissue is unknown, so it is called “idiopathic” (meaning without an obvious origin). “However, the body’s healing process plays an important role in scarring. When the lungs are injured—from environmental factors such as dust, smoke or infection, or possibly even from autoimmune reactions—the body tries to repair the damaged tissue. In a healthy response, this repair is controlled and temporary. However, in IPF, this repair process is disrupted, and the body overproduces collagen and other fibrous materials. Instead of normal lung tissue regeneration, excessive scar tissue forms, which progressively impairs lung function,” Dr. Modi says. This abnormal healing response leads to the irreversible scarring characteristic of IPF. The exact mechanism is still under investigation, but it is believed that genetic factors, environmental exposures, and chronic inflammation all play a role in triggering this defective repair process.
What are the symptoms?
Fibrosis hardens the lungs, making breathing difficult. “Symptoms include shortness of breath, dry cough, fatigue and weight loss. Over time, the amount of oxygen in the blood decreases, leading to complications such as pulmonary hypertension, heart attack or respiratory failure,” says Dr Bhasin.
Who is at risk?
Most cases occur in people over the age of 50. “IPF is slightly more common in men than women. Genetic predisposition may play a role in some cases. Current or ex-smokers are at higher risk. Prolonged exposure to dust, wood or metal particles can increase the risk,” explains Dr Bhasin. GERD patients, who have repeated subtle aspiration of stomach acid, can develop lung tissue damage over time,” adds Dr Modi.
How is IPF diagnosed?
A high-resolution CT scan, pulmonary function tests, and sometimes a lung biopsy help confirm the diagnosis. “But other causes of lung disease need to be ruled out,” says Dr Bhasin.
How is the situation managed?
Antifibrotic drugs such as pirfenidone and nintedanib slow the progression of the disease, Dr. Bhasin says. It is supplemented by oxygen therapy, lung exercises. “In advanced cases a lung transplant may be considered,” he says. So, says Dr. Modi, “
Early diagnosis and multidisciplinary care are critical.”
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