Is there any cure for Pulmonary Fibrosis? - Dr. Hirennappa B Udnur

The most important criteria in radiology is to make a difference between it is whether it is a UIP or a non UIP. If it is really an idiopathic pulmonary fibrosis, you normally don’t require to go with a biopsy and CT diagnosis is equivalent to a UIP pattern, which is a grave amount and the natural state of the disease is 3 to 5 years. Sometimes they deteriorate faster. Nothing was available earlier than the steroids and immunosuppressants and N-acetyl cysteine, which are used as antioxidants. As the guidelines moved on, we say that steroids must not be used in idiopathic pulmonary fibrosis. That should be except for during acute exacerbations of ILD’s. So we use anti fibrotic agent called as pirfenidone, which was introduced in 2012 and this delays the progression of the disease. The patient don’t feel the relief of breathlessness. They will still have some breathlessness. So it delays the progression of the disease. Apart from that, the treatment options for any kind of ILD, in general is supportive treatments like oxygenation might be required and the treatment of secondary pulmonary hypertension, we have PH lowering drugs like sildenafil analogues and tadalafil and sometimes and ambrisentan and bosentan can be used in these kind of situations and oxygenation, we need supplemental home oxygen therapy with oxygen concentrator, and during acute situations here oxygen requirement is very high, they might require a high flow systems in house if it is really advanced diseases and in early stages, we just give ambulatory oxygen therapy, where the pulse oximeter plays a huge important role and keeping this, patients managing fit will their their last minutes, otherwise they will be dependent on the house or dependent on somebody in oxygen dependent patients. Apart from antioxidants like N-acetyl cysteine are common to both and they say they can use that will prevent and vaccinations like pneumococcal vaccination and influenza vaccination can cause any preexisting lung disease they are prone for pneumonia and since they are in a tight balance and a poor revere of cardiorespiratory, they can worsen fast if they can sustain a small infections or apart from the routine one what we are giving sometimes associated smokers, if they have COPD or obstructive pattern, we still require a bronchodilator inhalers in them in them. Apart from that is most of the specific treated is antifibrotic. Now the latest kid in the block in nintedanib. That is a very costly drug and it is a triple thyrosin kinase inhibitors. They say that good results are there. Cost is a limiting factor, bit at is available in India. So the ultimate therapy for pulmonary fibrosis, which is more of idiopathic pulmonary fibrosis is lung transplant. Internationally they have big centers for lung transplant. They do a single lung transplant or a double lung transplant or even a heart lung transplantations can be used, but in India, the availability of lung transplant facilities overall is less. Since the access is, we are more prone to infections and bronchiolitis obliterans is a major complication. We need to think and approach what like in a renal transplant and a liver transplant, in the future I think lung transplant might be more common for idiopathic pulmonary fibrosis and in a non-idiopathic pulmonary fibrosis, usually it is like a nonspecific interstitial disease. There is more of a connective tissue disorder and we treat it with steroids and immunosuppressants like we treat any connective tissue disorders like rheumatoid arthritis, SLE and other things. So sometimes if it is an idiopathic or non-connective tissue disorders also, we resort to a lung biopsy and based on the lung biopsy, we give steroids and immnosuppresants like azathioprine and now the new safest immunosuppressants like mycophenolate mofetil can be tried. All other diseases, most of them are steroids and immunosuppressants. So we need to differentiate. One is the steroid dependent, where we should use the non UIP setting and another condition, UIP you can treat, in such conditions we don’t use much of steroids, except for the exacerbation. Any exacerbation, we still need steroids and the most important role of pulmonary hypertension, always needs to be explained pulmonary hypertensions can really prolong their life. We don’t have much in terms of medical treatment available in these kind of situations.