Pulmonary fibrosis and IPF: specialist care in Bangalore

Where this page fits

This page is about getting treated. If you are looking for a full explanation of what pulmonary fibrosis is, how it is diagnosed and what the outlook looks like, read the detailed patient guide, pulmonary fibrosis in India. What follows here is the clinical side: what treatment is actually available in India, what it costs, what the decision points are, and what happens when you come to the clinic.

Dr. Manjunath M Negigoudara

Transplant Pulmonologist, KIMS

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The four decisions that matter in fibrosis care

Most of what determines how a fibrosis patient does over the following years comes down to four decisions, and it is worth knowing what they are so you can ask whether each has been made.

1. Is the diagnosis right? Pulmonary fibrosis is a pattern, not a cause. Before accepting idiopathic, the treatable causes have to be excluded properly.
2. Should antifibrotic treatment start, and when? The evidence supports starting once a progressive fibrotic pattern is established, rather than waiting for significant decline.
3. What else is making the breathlessness worse? Reflux, sleep apnoea, pulmonary hypertension, deconditioning and anaemia all add to breathlessness and all are treatable.
4. Where does this patient stand on transplant? Asked early, this question is planning. Asked late, it is often too late to act on.

Is my pulmonary fibrosis really idiopathic?

Idiopathic means no cause has been found. It does not mean no cause exists, and a meaningful number of patients labelled idiopathic turn out to have something identifiable and more treatable underneath. Before the label is accepted, three things need to have been done thoroughly.

A complete autoimmune panel, because fibrosis is sometimes the first manifestation of rheumatoid arthritis, scleroderma, myositis or Sjogren syndrome, and connective-tissue-disease fibrosis is treated differently. A forensic exposure history, because chronic hypersensitivity pneumonitis can look identical to IPF on a scan and is driven by something in the patient's environment, most often birds, damp and mould, or feather bedding. And an HRCT read to protocol, with prone and expiratory images, because air trapping on the expiratory images points away from IPF and toward hypersensitivity pneumonitis.

Where the picture remains unclear, the case goes to a multidisciplinary discussion between the pulmonologist, a thoracic radiologist and a pathologist. This is the reference standard in international guidelines and it changes the working diagnosis often enough to justify the effort. The full diagnostic pathway is set out on the interstitial lung disease page.

What is the treatment for pulmonary fibrosis in India?

Two drugs are established for idiopathic pulmonary fibrosis: nintedanib and pirfenidone. Both slow the rate at which lung function declines, nintedanib in the INPULSIS trials and pirfenidone in the ASCEND trial, both published in 2014. Neither reverses scarring, and neither has been shown to be superior to the other, so the choice between them is made on side effects and on what an individual patient will tolerate and keep taking.

The India-specific point that matters most: both drugs are manufactured as Indian generics, which places the monthly cost at a small fraction of the originator price in Western markets. This is the single biggest practical advantage Indian fibrosis patients have, and it is the reason affordability is discussed openly in the clinic rather than avoided. An antifibrotic that a patient stops after two months because of cost has done nothing for them.

Antifibrotic treatment is not restricted to IPF. Where fibrosis of another cause behaves in a progressive fibrotic way, the same drugs are used alongside whatever treats the underlying disease. This progressive fibrosing phenotype is now a recognised treatment indication in its own right, established by the INBUILD trial and carried into the 2022 ATS/ERS/JRS/ALAT guideline.

What else is making my breathlessness worse?

Patients and doctors both tend to attribute all breathlessness to the fibrosis itself. Often a meaningful part of it is coming from something else, and that part is treatable.

• Gastro-oesophageal reflux is very common in fibrosis, frequently silent, and suspected of contributing to ongoing lung injury through micro-aspiration.
• Obstructive sleep apnoea is common and under-diagnosed in this group. Treating it improves daytime energy and night-time oxygen levels. Assessment is available through the sleep medicine service.
• Pulmonary hypertension develops in advanced fibrosis and changes both prognosis and management. It is assessed in the same clinic. See pulmonary hypertension.
• Deconditioning compounds itself, as breathlessness reduces activity and reduced activity worsens breathlessness. Pulmonary rehabilitation reliably breaks this cycle.
• Exertional hypoxia is missed whenever oxygen is only checked at rest. Ambulatory oxygen for those who desaturate on walking restores a great deal of independence.

When does pulmonary fibrosis need a lung transplant?

Severity is staged with the GAP index, which combines gender, age and lung function, and is worth asking your specialist about because it frames how urgent the transplant question is.

Idiopathic pulmonary fibrosis is the leading indication for lung transplantation worldwide, and fibrosis patients are the group most often referred too late. The reason is that fibrosis can progress unpredictably. A patient can be stable for a year and then lose substantial lung function in a few months, or after a single acute exacerbation.

The ISHLT 2021 consensus on candidate selection is to refer at diagnosis for an otherwise suitable IPF patient. Referral means an assessment and a conversation, nothing more. Patients assessed early and found suitable are then simply followed, with a clear plan for what triggers listing. Patients who arrive already on continuous oxygen, already deconditioned, or during an acute exacerbation, frequently cannot complete the assessment at all.

Dr. Manjunath M Negigoudara performs this assessment at KIMS Electronic City. Read about lung transplantation, the cost of lung transplant in Bangalore, and life after a lung transplant.

What is an acute exacerbation of pulmonary fibrosis?

An acute exacerbation is a sharp worsening of breathlessness over days to a few weeks, with new shadowing on imaging and no alternative explanation, as defined by the international working group report. It is the most dangerous event in the course of fibrosis and it needs to be treated as an emergency rather than as a routine deterioration.

Seek urgent care if your breathlessness has clearly worsened over days rather than months, if you need oxygen when you did not before, if you have a new fever or cough with sputum, or if your resting oxygen saturation has fallen below your usual reading. Call +91 79937 41199 or attend the emergency department at KIMS Electronic City. Where respiratory failure is severe and the underlying lungs may recover or a transplant may be possible, ECMO is available on site.

What happens at a pulmonary fibrosis consultation in Bangalore?

Bring the HRCT images themselves, not only the report, every previous pulmonary function test so the trend can be seen, your autoimmune blood results, and a complete list of current medicines including doses. Expect a detailed exposure history, an examination, a six-minute walk test with oximetry, and a plain statement of the diagnosis, the treatment plan and where you stand on transplant.

KIMS Hospital, Survey No. 37 and 38, PES University EC Campus, Hosur Road, Konappana Agrahara, Bengaluru 560100. Monday to Saturday, 9 AM to 5 PM, with evening and video consultations by prior appointment. Call or WhatsApp +91 79937 41199.

For patients from Bangalore, across India, and abroad

Bengaluru and Electronic City

The hospital is on Hosur Road opposite the PES University EC Campus, about 15 minutes from Silk Board via the Electronic City elevated expressway. Fibrosis patients attend from HSR Layout, Koramangala, Sarjapur Road, BTM Layout, Jayanagar, JP Nagar, Bannerghatta Road, Whitefield and Marathahalli, and from Bommasandra, Anekal, Chandapura, Attibele and along Hosur Road to the south.

Karnataka and the rest of India

Patients travel from Mysuru, Mangaluru, Hubballi-Dharwad, Belagavi, Kalaburagi, Shivamogga, Tumakuru, Davangere and Ballari, and from Chennai, Coimbatore, Madurai, Tiruchirappalli, Salem, Hyderabad, Warangal, Vijayawada, Visakhapatnam, Tirupati, Guntur, Kochi, Thiruvananthapuram, Kozhikode, Kannur, Goa, Mumbai, Pune, Nagpur, Ahmedabad, Delhi NCR, Kolkata, Bhubaneswar, Guwahati, Chandigarh, Lucknow, Patna, Ranchi and Raipur. Send your HRCT and pulmonary function reports by WhatsApp before travelling so the visit can be planned around what is already known. Kempegowda International Airport is about 45 km from the hospital.

International patients

Fibrosis patients are seen from Bangladesh, Nepal, Sri Lanka, the Maldives and Bhutan, from Oman, the UAE, Saudi Arabia, Iraq, Yemen, Kuwait, Bahrain and Qatar, from Kenya, Tanzania, Ethiopia, Uganda, Rwanda, Sudan, Nigeria and Ghana, and from Myanmar, Indonesia and Malaysia. The pathway is a remote review of your imaging and reports over WhatsApp, a written clinical opinion that can support a medical visa application, a three to four day evaluation visit, and video follow-up afterwards. Indian generic antifibrotics are a substantial part of why patients from these regions choose to be worked up here.

Related reading

• Pulmonary fibrosis in India, the full patient guide
• Interstitial lung disease, the wider family of conditions and the diagnostic pathway
• Pulmonary hypertension, a common complication of advanced fibrosis
• Post-COVID lung damage, where fibrosis follows infection
• Lung transplant cost in Bangalore
• Second opinion on a fibrosis diagnosis
• All lung conditions treated at KIMS Electronic City