Interstitial Lung Disease and Pulmonary Fibrosis

Interstitial Lung Disease and Pulmonary Fibrosis

S.K. Jindal,

MD (Medicine), FAMS, FCCP, FNCCP

(Former Professor & Head, Department of Pulmonary Medicine,

 PGIMER, Chandigarh, India)

Medical Director

Jindal Clinics, SCO 21, Sector 20 D, Chandigarh, India 160020

Interstitial lung disease (ILD) is a group of over 200 lung diseases in different types of medical problems which involve the lung parenchyma, the interstitium.  It is a heterogeneous group of diverse causes.  These diseases are listed as a single entity (i.e. ILD) because the clinical presentation and chest radiography are mostly similar.  Moreover, the differential diagnosis of individual diseases is generally difficult.

Pulmonary fibrosis (PF), or more precisely idiopathic pulmonary fibrosis (IPF) is the most serious form of ILD.  Routinely, one tends to use the terms ILD and IPF interchangeably.  As stated earlier, the two terms have different connotations.

IPF is being increasingly diagnosed these days.  There is no known cause of IPF.  That is why, it puzzles the patient and his/her relatives who repeatedly question:  “ Why has it happened?”  “Why the problem cannot be cured, or at least its progression stopped?”  Unfortunately, the disease can neither be cured, nor permanently arrested.  It continues to progress at variable speed even with different treatments.

An IPF patient will commonly complain of dry cough and/or breathlessness which continue to progress relentlessly.  Both the symptoms are highly annoying, distressing and troublesome.  They do not respond to routine treatments for cough and breathlessness.  Not infrequently, the patient is unable to sleep and rest because of the unrelieved cough.  Breathlessness in the earlier stages appears only on exertion or exercise.  In late stages, patient gets breathless on even routine daily activities such as taking a bath or moving out of the bed. 

Most of the symptoms are attributable to shrinkage of the lungs due to fibrosis (i.e. scarring) of the lung parenchyma.  There is decreased lung capacity due to marked reduction in lung size.  The respiratory reserve get markedly reduced. In due course of time, there is onset of decreased oxygen saturation and respiratory failure.

Diagnosis of IPD is made on the basis of clinical history, examination and chest radiography which includes a good high resolution CT scan of the lungs.  Blood investigations and lung biopsy are sometimes required.

Treatment of IPF in the early stages is given with anti-fibrotic and other supportive drugs. The treatment is aimed to slow down the progression.  Not much is achieved with this treatment in the late stages when supportive therapy with supplemental oxygen administration at home and other drugs may help to alleviate some of the symptoms.  Unfortunately, the advanced disease becomes difficult to handle. 

Lung transplantation is the only answer in such patients.  The facility for lung transplantation is very limited in this country largely because of the non-availability of donors.

We can only hope that a good treatment will soon emerge and the lung transplantation facility becomes widely available.  This can happen only if the people realize the importance and social responsibility to donate their organs for the needy at their unfortunately demise.