As Zakir Hussain, tabla maestro and five-time Grammy Award winner, passed away on Sunday in San Francisco due to idiopathic pulmonary fibrosis (IPF), there are concerns as to how this happens and the age group vulnerable to it.
According to Dr Deepak Bhasin, Senior Director, Pulmonology, Critical Care, Max Hospital, Mohali, “It is a chronic, progressive lung disease characterised by scarring (fibrosis) of the lung tissue, leading to irreversible loss of lung function. The exact cause of IPF is unknown, so it is termed idiopathic.” This scarring thickens and stiffens the lungs, reducing their ability to expand and take in oxygen. “Unlike many lung conditions, IPF specifically targets the interstitium, the tissue surrounding the air sacs (alveoli), making it harder for oxygen to move into the bloodstream. Over time, this leads to persistent breathlessness, fatigue, and reduced quality of life,” adds Dr Mahavir Modi, pulmonologist and sleep specialist, Ruby Hall Clinic, Pune.
Why does scarring happen in IPF?
The exact cause of the initial injury to lung tissue is unknown, which is why it is called “idiopathic” (meaning without a clear origin). “However, the body’s healing process plays a critical role in scarring. When the lungs experience injury — due to environmental factors like dust, smoke, or infections, or possibly even from autoimmune responses — the body attempts to repair the damaged tissue. In a healthy response, this repair is controlled and temporary. However, in IPF, this repair process becomes dysregulated, and the body overproduces collagen and other fibrous materials. Instead of normal lung tissue regenerating, excessive scar tissue forms, which progressively impairs lung function,” says Dr Modi. This abnormal healing response leads to irreversible scarring characteristic of IPF. The exact mechanism is still under research, but it is believed that genetic factors, environmental exposures, and chronic inflammation all play a role in triggering this faulty repair process.
What are symptoms?
Fibrosis causes the lungs to stiffen, making breathing harder. “Symptoms include shortness of breath (dyspnea), dry cough, fatigue, and weight loss. Over time, oxygen levels in the blood drop, causing complications such as pulmonary hypertension, heart failure, or respiratory failure,” says Dr Bhasin.
Who is at risk?
Most cases occur in people over 50 years. “IPF is slightly more common in men than women. A genetic predisposition may play a role in some cases. Current or former smokers have a higher risk. Prolonged exposure to dust, wood, or metal particles may increase the risk,” explains Dr Bhasin. “Sometimes patients of chronic GERD, who have repeated micro-aspirations of stomach acid, may suffer damage in lung tissue over time,” adds Dr Modi.
How is IPF diagnosed?
High-resolution CT scans, pulmonary function tests, and sometimes lung biopsy help confirm the diagnosis. “But it is essential to rule out other causes of lung diseases,” says Dr Bhasin.
How is the condition managed?
Antifibrotic drugs like pirfenidone and nintedanib slow the disease progression, says Dr Bhasin. This is complemented by oxygen therapy, lung exercises. “Lung transplant may be considered for advanced cases,” he says. That’s why, says Dr Modi, “
Early diagnosis and multidisciplinary care are critical.”
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