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Pulmonary fibrosis and IPF: specialist care in Bangalore

Scarring of the lung is permanent, but the rate at which it progresses is not fixed, and a great deal of what determines that rate is within reach of treatment. Dr. Manjunath M Negigoudara treats pulmonary fibrosis and idiopathic pulmonary fibrosis at KIMS Hospital, Electronic City, Bengaluru, with transplant assessment in the same department.

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.

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.

Decision one: is it 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.

Worth asking at your next appointment

Has my autoimmune panel been done and was it normal? Was my HRCT done with prone and expiratory images? Has anyone asked me in detail about birds, damp or my workplace? If the answer to any of these is no, the diagnosis has not been fully worked up.

Decision two: antifibrotic treatment

Two drugs are established for idiopathic pulmonary fibrosis: nintedanib and pirfenidone. Both slow the rate at which lung function declines. 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.

Nintedanib Pirfenidone
Main side effectDiarrhoea, usually manageable with dose adjustment and antidiarrhoealsNausea, rash and marked sun sensitivity
MonitoringLiver function testsLiver function tests
Practical notesTaken twice daily with food. Interacts with anticoagulants.Taken three times daily with food. Strict sun protection needed.
Also used inProgressive fibrosing ILD of other causes, and scleroderma-associated ILDPrimarily IPF

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.

Decision three: the things that make 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.

Decision four: where you stand on transplantation

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.

International consensus 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.

Recognising an acute exacerbation

An acute exacerbation is a sharp worsening of breathlessness over days to a few weeks, with new shadowing on imaging and no alternative explanation. 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 a fibrosis consultation involves

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

Frequently asked questions

Who is the best pulmonary fibrosis specialist in Bangalore?
Pulmonary fibrosis should be managed by a pulmonologist who sees it in volume and who can also answer the transplant question, because that question arrives for most fibrosis patients eventually. Dr. Manjunath M Negigoudara is a transplant pulmonologist at KIMS Hospital, Electronic City, Bengaluru, and has personally managed 250+ lung transplant cases within the KIMS Institute of Heart and Lung Transplant, which has completed 780+ thoracic transplants. Call or WhatsApp +91 79937 41199, Monday to Saturday, 9 AM to 5 PM.
Are nintedanib and pirfenidone available in India, and what do they cost?
Both antifibrotic drugs are available in India, including as Indian generics, which places them at a small fraction of what the same drugs cost in the United States or Europe. Your treating pulmonologist will choose between them based on side-effect profile rather than efficacy, since neither has been shown superior to the other. Cost is discussed openly in the clinic, because these are long-term daily medicines and affordability decides whether a patient actually stays on treatment.
Can pulmonary fibrosis be reversed?
Established scarring cannot currently be reversed by any available treatment. That is the honest answer and patients deserve it plainly. What can be done is substantial: antifibrotic therapy slows the rate of decline, treating the conditions that travel alongside fibrosis improves how you feel day to day, pulmonary rehabilitation improves walking distance, and for suitable patients lung transplantation replaces the scarred lung entirely. Not reversible is not the same as untreatable.
Is idiopathic pulmonary fibrosis hereditary?
Most cases are not. A minority of patients have a familial form, and the clue is a family history of pulmonary fibrosis in a parent or sibling, or fibrosis appearing at an unusually young age. Where that history exists it is worth telling your pulmonologist, because it changes how closely relatives should be watched and occasionally points toward a specific genetic cause.
What is an acute exacerbation of pulmonary fibrosis?
A sudden worsening of breathlessness over days to weeks, with new shadowing on the scan and no other explanation such as infection, clot or heart failure. It is the most feared complication of fibrosis and it is a medical emergency. If your breathlessness changes sharply over a few days, do not wait for the next scheduled appointment. Call +91 79937 41199 or attend the emergency department at KIMS Electronic City.
When should a pulmonary fibrosis patient be referred for lung transplant?
Earlier than most patients are told. International consensus is to refer at diagnosis for idiopathic pulmonary fibrosis in an otherwise suitable patient, rather than waiting for deterioration. Fibrosis is unpredictable and the assessment itself takes weeks. Referral simply starts a conversation and an assessment. It does not commit you to surgery. See who needs a lung transplant.
Does oxygen therapy help in pulmonary fibrosis?
For patients whose oxygen levels fall on exertion, yes, and it is frequently under-prescribed. Ambulatory oxygen lets people walk further and do more, which protects muscle strength and mood. The test that establishes the need is a six-minute walk with continuous oximetry, not a resting reading, because resting saturation can look entirely normal in someone who drops sharply on walking.
Can I get a second opinion on my fibrosis diagnosis without travelling?
Yes. WhatsApp the HRCT images, the pulmonary function report including DLCO, your autoimmune blood results and your current medication list to +91 79937 41199. Dr. Manjunath issues a written opinion covering whether the diagnosis fits the imaging, whether an alternative diagnosis deserves consideration, whether antifibrotic treatment is indicated, and where you stand on transplant. See the second opinion page.
Dr. Manjunath M Negigoudara - Transplant Pulmonologist, KIMS Electronic City
Dr. Manjunath M Negigoudara Transplant Pulmonologist · KIMS Hospital, Electronic City, Bengaluru