New Delhi: Pulmonary fibrosis is a lung disease characterised by inflammation and scarring (fibrosis) of lung tissue, making it increasingly difficult to breathe. The scarring causes the lungs to become stiff, hindering their ability to absorb oxygen into the bloodstream. This condition encompasses approximately 200 clinical interstitial lung diseases that involve inflammation and scarring around the alveoli.
While talking about pulmonary fibrosis, Dr Ishaan Capoor, Consultant Pulmonologist at Narayana Health City, answered all FAQs about the condition.
Each September, we observe Pulmonary Fibrosis (PF) Awareness Month to promote understanding of this disease. With the theme “Every Breath, Every Story,” the Pulmonary Fibrosis Foundation highlights patient journeys, encouraging families and supporters to raise awareness. The most common form of pulmonary fibrosis is idiopathic pulmonary fibrosis (IPF) which doesn’t have any definitive cause. Current and former smokers are at a higher risk of developing IPF as compared to those who have never smoked. Age is another factor, as IPF is rare before the age of 50. Certain types of pulmonary fibrosis can run in families.
Other risk factors include older age, male sex, and exposure to dust and fumes. In recent years, cases in women have also been on the rise. Individuals in occupations such as farming, hairdressing, stone cutting, and metalworking may face an increased risk. Dust from feathers and bird droppings contain protein antigen which causes inflammation of the lungs when inhaled. Additionally, autoimmune conditions (in which the patient’s immune system acts against itself )such as Rheumatoid Arthritis and Scleroderma can lead to pulmonary fibrosis. Pulmonary Fibrosis may also occur post viral pneumonia, including post-severe COVID-19 and H1N1 pneumonia. Furthermore, radiation therapy and certain chemotherapy agents can contribute to the development of this disease.
Patients with pulmonary fibrosis may experience symptoms such as breathlessness, dry cough, fatigue upon exertion, chest tightness, decreased appetite, and weight loss. Early detection and diagnosis are critical so that appropriate steps can be taken to slow down the progression of the disease. They may also exhibit cyanosis – bluish discoloration of the skin and nail beds due to low oxygen levels in the blood, along with clubbing—thickening of the tissue under the fingernails that causes the nails to curve downward.
Diagnosis typically involves a chest X-ray and CT Chest imaging to rule out other lung-related illnesses. Pulse oximetry monitoring, both at rest and during a six-minute walking test, provides additional information about the patient’s condition and disease progression. Pulmonary function tests help monitor lung function and document any decline. If left untreated, pulmonary fibrosis can lead to increased strain on the right side of the heart, resulting in elevated blood pressure in the lungs and potential right-sided heart failure.
Currently, there is no cure for pulmonary fibrosis, but there is light at the end of the tunnel as treatment options are available to improve the quality of life for those affected. These options include antifibrotic medications and other medications that suppress Inflammation namely corticosteroids and immunosuppressive agents, oxygen therapy, and pulmonary rehabilitation. Patients with Pulmonary Fibrosis should be advised of vaccination as a preventive measure against Pneumonia and Influenza Infection. In severe cases, a lung transplant may be necessary, requiring thorough assessment and evaluation for eligibility.
By raising awareness and understanding of pulmonary fibrosis, we can support those living with this challenging condition and advocate for continued research and treatment advancements.
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