Pulmonary Fibrosis: Recognizing Symptoms and Management

Pulmonary fibrosis is a condition characterized by the formation of scar tissue (fibrosis) that causes the lungs to become stiff and lose their elasticity. The causes of pulmonary fibrosis are varied, ranging from long-term exposure to harmful substances such as silica dust, asbestos, and industrial chemicals, to autoimmune diseases such as rheumatoid arthritis, as well as side effects of treatments like chemotherapy and radiotherapy. There is also a form of pulmonary fibrosis called idiopathic pulmonary fibrosis (IPF), where the cause is unknown, and the disease is progressive with a poor prognosis.

Symptoms of pulmonary fibrosis usually develop gradually and often go unnoticed. The main symptoms to watch for include worsening shortness of breath, especially during activity, a persistent dry cough, fatigue, unexplained weight loss, and changes in the shape of the fingertips (clubbing). If these symptoms occur, pulmonary fibrosis should be considered as a possible diagnosis.

Diagnosis of pulmonary fibrosis is made through clinical evaluation, pulmonary function tests showing a restrictive pattern, and radiological examination with high-resolution CT scan (HRCT) that reveals characteristic patterns of fibrosis and scarring in the lungs. In some cases, lung biopsy is also necessary to confirm the diagnosis and rule out other causes.

To date, pulmonary fibrosis cannot be completely cured because the scar tissue that has formed is permanent. However, treatment aims to slow disease progression and improve patient’s quality of life. Available therapies include antifibrotic drugs such as pirfenidone and nintedanib, which have been shown to slow lung tissue damage, supplemental oxygen for patients with hypoxemia, and pulmonary rehabilitation to improve respiratory capacity and physical endurance. In very severe cases, lung transplantation may be an option.

Early detection of pulmonary fibrosis is crucial so that treatment can be started promptly and achieve optimal outcomes. Unfortunately, because initial symptoms are nonspecific, many patients are diagnosed late. Awareness of persistent cough and shortness of breath, especially among those with risk factors such as exposure to harmful substances at work, history of autoimmune diseases, or previous chronic lung diseases, is essential.

Prevention of pulmonary fibrosis can be achieved by avoiding exposure to harmful substances, using protective equipment when working in high-risk environments, quitting smoking, and avoiding exposure to cigarette smoke. Regular lung health check-ups are also strongly recommended for at-risk groups.

In summary, pulmonary fibrosis is a chronic progressive disease; however, with good understanding, awareness of symptoms, and appropriate medical intervention, patients can slow lung damage progression and maintain a better quality of life.

References:

1.     Experimental anticancer drug shows promise as treatment for Idiopathic Pulmonary Fibrosis. National Heart, Lung, and Blood Institute (NHLBI), NIH. 2022. https://www.nhlbi.nih.gov/news/2022/experimental-anticancer-drug-shows-promise-treatment-idiopathic-pulmonary-fibrosis

2.     Pulmonary Fibrosis - Treatment. National Heart, Lung, and Blood Institute (NHLBI), NIH. 2022. https://www.nhlbi.nih.gov/health/idiopathic-pulmonary-fibrosis/treatment

3.     Idiopathic Pulmonary Fibrosis (IPF) Guidelines: Guidelines Summary. Medscape. https://emedicine.medscape.com/article/301226-guidelines

4.     Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis. Archivos de Bronconeumología. 2022. https://www.archbronconeumol.org/en-estadisticas-S1579212913001067

5.     Guidelines of the Polish Respiratory Society for Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis. Advances in Respiratory Medicine. 2020. https://www.mdpi.com/2543-6031/88/1/42