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Idiopathic Pulmonary Fibrosis: An Interstitial Lung Diseases That Cannot Be Reversed
Idiopathic Pulmonary fibrosis (IPF) is a subset of interstitial lung diseases, where in the normal ageing of lung is augmented. It is characterised by fibrosis of the lung architecture which results in reducing the oxygen and carbon-di-oxide exchange capacity of the lung leading to respiratory failure.
As the name suggest 'Idiopathic' there is no exact cause identified. In few percentages, it may be inherited in a family. Most often, smoking is a contributory factor.
Lung, as we know is the air filter of our body, it plays a crucial role in supplying oxygen to the body and gets rid of toxic gases, predominantly carbon-di-oxide, which are vital for survival. Being a huge organ, these vital functions are usually preserved until 70% of the lung functions are affected. This usually leads to delayed presentation and diagnosis. Early manifestations of IPF are dry cough not relieved by primary line of cough treatment. Breathlessness is usually masked with sedentary lifestyle, it only manifests on exertion. In IPF, symptoms are usually progressive in nature.
IPF is a serious condition, and you need to be aware of certain facts of this disease.
Late presentation: Usually, IPF is diagnosed in later stages of the disease. Several factors contribute for delayed diagnosis, chronic cough is usually managed by home remedies and at primary level of medical care. Breathlessness again may not be perceived as a warning sign unless an individual is involved in exertional activities. A chest Xray imaging is a poor tool in early stages of diagnosis.
Delayed diagnosis: A High Resolution Computed Tomography scan (HRCT) of the chest is the most sensitive tool currently available for diagnosis of IPF. An early level of suspicion of IPF and timely scan helps in early diagnosis. Its availability, cost of scan and radiation risk are a few common hurdles why they may not be prescribed to patients.
Unavailability of full proof treatment: As of now, we do have any medical management which can reverse lung fibrosis or can improve the affected lung functions. Anti-fibrotic drugs do have a role in few select patients which helps delay the progression of the diseases and stabilizing lung functions. Lung transplant is the only treatment, which after carefully selecting the right patients can be offered in IPF, to get rid of the disease.
Early diagnosis is the key. Due to lack of any corrective treatment the entire focus is on the preventive aspect.
If you want to delay the progression of the disease and improve your quality of life, you need to do the following
In suitable patients, lung transplant is the corrective treatment.
If you have the following symptoms, you need to visit a pulmonologist immediately.
(This article is authored by Dr. Vaibhav R. Kubal Consultant- Respiratory Medicine at Holy Family Hospital, Bandra, Mumbai)