Comprehensive review of potential drugs with anti-pulmonary fibrosis properties



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What Is Idiopathic Pulmonary Fibrosis? Know More About Cause Of Tabla Maestro Zakir Hussain's Death

Renowned musician Ustad Zakir Hussain passed away on Sunday night following complications with idiopathic pulmonary fibrosis. Tributes have poured in from across the globe following the news of his demise.

According to a statement released by the family, Hussain died from complications arising out of idiopathic pulmonary fibrosis. The tabla maestro had been hospitalised in San Francisco for the past two weeks, and was shifted to the intensive care unit after his condition deteriorated.

"He passed away very peacefully after the ventilation machine was switched off. This was 4 pm San Francisco time," his sister Khurshid Aulia told PTI.

What is idiopathic pulmonary fibrosis?The US National Heart, Lung and Blood Institute defines it as a "serious chronic disease that affects the tissue surrounding the air sacs, or alveoli, in the lungs". The lung tissue becomes thick and stiff for unknown reasons — causing permanent scarring in the lungs over time. Fibrosis can makes it progressively more difficult to breathe for the patient.

Symptoms develop and worsen as the disease progresses — with higher risk for smokers or those who have a family history of IPF. The risk of idiopathic pulmonary fibrosis also increases with age. Common symptoms include shortness of breath and a cough. Many people with IPF also experience acute exacerbations — wherein symptoms suddenly become much more serious.

Complications from IPF include pulmonary hypertension and respiratory failure, which happen when the lungs cannot deliver enough oxygen into the bloodstream without support. This prevents the brain and other organs from getting the oxygen they need.

What are the symptoms?Common symptoms include shortness of breath and a dry cough — both of which are likely to get worse over time. Patients might also have aching muscles and joints as well as feeling extreme tiredness. They may notice a gradual, unintended loss of weight and generally feel unwell. Symptoms also include rapid, shallow breathing and clubbing — a widening and rounding of the tips of the fingers or toes.


Tabla Legend Zakir Hussain Passes Away At 73, Know How Idiopathic Pulmonary Fibrosis Can Be Fatal

What Is Idiopathic Pulmonary Fibrosis?

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Dr. Harpalsinh Dabhi, Senior pulmonologist and critical care Physician, HCG Hospitals, Bhavnagar, says, "Idiopathic Pulmonary Fibrosis (IPF) is a chronic and progressive lung disease marked by scarring (fibrosis) of lung tissue, which gradually makes breathing more difficult. While the exact cause is unknown, it is believed to result from a combination of genetic, environmental, and age-related factors. The condition primarily affects adults over the age of 50, with the risk increasing as people age. Idiopathic Pulmonary Fibrosis is like living with lungs that slowly lose their ability to expand and contract, turning every breath into a challenge."

"Idiopathic" means the cause is unknown, which makes the condition even worse and thus incurable. There is no clear trigger, and it only gets worse with time.

How Does IPF Manifest?

Our expert, Dr. Bhimasena Rao R S, Senior Consultant Pulmonology, Fortis Hospital Rajajinagar, Bangalore explains that patients commonly report a gradual onset (over several months) of dyspnea on exertion and a nonproductive cough. Fatigue, fever, myalgias, and arthralgias are rarely reported. Bibasilar crackles are usually audible on physical examination but may be absent or heard unilaterally in the early stages of the disease. Additionally, clubbing (deformation of the nail base) is a manifestation of advanced IPF.

Who Is At Risk?

According to Dr Rao, the onset of IPF typically occurs in patients aged 60 years and older, except in those with familial pulmonary fibrosis (FPF), where the disease presents earlier. Male patients appear to be at increased risk, and the majority of patients have a history of cigarette smoking. Hussain's untimely death is a stark reminder that even the strongest, most disciplined individuals are not always immune to such devastating illnesses.

How Is IPF Diagnosed?

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"Diagnosis involves a thorough history, environmental exposure assessment, high-resolution CT (HRCT) scans, pulmonary function tests (PFTs), and blood tests to rule out other causes of interstitial lung disease (ILD). The diagnosis of IPF requires the exclusion of other known causes of ILD and the presence of either definite or probable features of UIP on HRCT. Multidisciplinary discussion (MDD) involving specialists from pulmonary, pathology, radiology, and sometimes rheumatology improves diagnostic accuracy and is considered the gold standard", explains Dr Rao.

RELATED: Maggie Smith, Beloved Star of Downton Abbey and Harry Potter, Passes Away at 89

Comorbidity And IPF Complications

IPF doesn't just damage the lungs; it merges with innumerable other problems that worsen the affected individual's quality of life and deteriorate their condition further. A review by PubMed Central lists the following complications and comorbid conditions.

Pulmonary Hypertension: Scarring in the lungs makes the heart work harder to pump blood, often leading to heart failure.

Lung Cancer: Those with IPF are up to five times more likely to develop lung cancer.

Chronic Respiratory Failure: As breathing becomes harder, the body slowly loses its ability to function properly.

Sleep Disorders: Oxygen levels drop dangerously low during sleep, leaving patients exhausted and drained.

Gastroesophageal Reflux: Stomach acid can enter the lungs, worsening the damage over time.

Additionally, Dr Rao shares that the differential diagnosis of IPF also includes other diseases with histopathologic features of UIP, such as rheumatic diseases (e.G., rheumatoid arthritis [RA], systemic sclerosis), chronic hypersensitivity pneumonitis, asbestosis, and certain drug-induced lung diseases. 

Why We Must Care About IPF?

IPF remains under-recognised, often misdiagnosed until it is too late. Dr Rao highlights that most cases of IPF progress despite treatment. Management strategies include oxygen therapy, steroids in some cases, antifibrotic drugs, pulmonary rehabilitation, and other supportive care measures, all aimed at slowing disease progression and improving quality of life. 

Lung transplantation may be considered for some younger IPF patients. UIP-IPF significantly reduces the survival of affected individuals. The road for IPF patients is eclipsed with uncertainty and challenge.

A Heartbreaking Farewell

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As we bid farewell to Zakir Hussain, let us not only remember his music but also shine a light on the disease that took him from us. His life is a poignant reminder of  the preciousness of health, and the urgency of raising awareness.

The legend's family rightly said in a statement that they gave to an Indian media outlet, "His prolific work as a teacher, mentor and educator has left an indelible mark on countless musicians. He hoped to inspire the next generation to go further. He leaves behind an unparalleled legacy as a cultural ambassador and one of the greatest musicians of all time".


Mortality Rates And Comorbidities Associated With Idiopathic Pulmonary Fibrosis And Progressive Pulmonary Fibrosis

Anna Podolanczuk, M.D., M.S., discusses how the mortality rates for patients with idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are impacted by comorbidities, including the role of autoimmune diseases in the pathophysiology of these conditions.

Video content above is prompted by the following:

  • What are the mortality rates for patients with IPF and PPF?
  • What are the comorbidities associated with IPF and PPF? How do autoimmune diseases contribute to the pathophysiology?




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