Pulmonary fibrosis: What is it and its long-term effects? - Dr Vikas Mittal

Pulmonary fibrosis is a chronic and progressive lung condition in which the normal tissue of the lungs becomes inflamed and gradually replaced with scar tissue, a process known as fibrosis.

This scarring makes the lungs stiff and thickened, impairing their ability to transfer oxygen into the bloodstream. As a result, patients often experience reduced oxygen levels in their blood, which leads to many of the distressing symptoms associated with the disease.

The most common problems faced by patients with pulmonary fibrosis include shortness of breath, particularly during exertion, a persistent dry cough, fatigue, low oxygen saturation (SpO₂ levels), and, in some cases, unexplained weight loss and swelling of the feet.

In advanced stages, patients may also develop clubbing of the fingertips, where the nails take on a rounded appearance due to long-term oxygen deprivation.

There are nearly 200 different types of pulmonary fibrosis, and in many cases, the exact cause cannot be identified. These are grouped under the term Idiopathic Pulmonary Fibrosis (IPF), which is considered one of the most severe forms of interstitial lung disease.

However, some known causes include connective tissue diseases such as rheumatoid arthritis, Sjögren’s syndrome, or scleroderma. Environmental and occupational exposures also play a role; inhaling organic dust (such as mould, pigeon droppings, or feathers) or industrial dusts (like silica and asbestos) can contribute to the development of fibrosis.

Certain medications, long-term smoking, air pollution, and even chronic acid reflux have also been recognised as risk factors.

Unfortunately, pulmonary fibrosis is usually progressive and irreversible. Some patients may experience a slow decline, while others deteriorate more rapidly.

Over time, the disease may lead to complications such as pulmonary hypertension (increased blood pressure in the lungs), which further strains the heart and can cause swelling in the lower limbs.

When it comes to treatment, there is no permanent cure for pulmonary fibrosis. However, advances in medicine have brought some relief through antifibrotic drugs like pirfenidone and nintedanib, which can slow the progression of scarring.

Supportive therapies such as oxygen supplementation and pulmonary rehabilitation play a crucial role in improving quality of life. In advanced cases where the disease continues to progress despite treatment, lung transplantation may be considered as a last resort.

Pulmonary fibrosis is a serious, life-altering illness that significantly impacts daily activities and overall well-being. Early recognition, timely diagnosis, and comprehensive management can help patients maintain a better quality of life and improve survival outcomes despite the challenges posed by this condition.

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