Pulmonary Fibrosis (PF) is a respiratory condition characterized by the thickening, stiffening, and scarring (fibrosis) of lung tissue. This scarring process makes it progressively difficult for the lungs to function efficiently and can lead to difficulty breathing, persistent cough, and decreased oxygen levels in the blood. Learn more about what pulmonary fibrosis is, what causes it, and how it’s treated.*

What is pulmonary fibrosis? 

The lungs are vital organs, continuously exchanging oxygen and carbon dioxide to keep our body functioning. However, in individuals with PF, this exchange becomes compromised due to the scarred tissues, leading to various symptoms and complications. 

According to David Lederer, M.D., Medical Director of Clinical Sciences, Respiratory and Inflammation at Regeneron Pharmaceuticals, lung disease encompasses many different disorders such as COPD, asthma and cystic fibrosis, in addition to over 100 types of interstitial lung diseases (ILDs). 

All ILDs share two features: inflammation and scar tissue, which arise in the walls of the lungs’ alveoli, or air sacs, where gas transfer occurs. Whereas these walls are normally 10 times thinner than a human hair, inflammation and scarring thicken them, inhibiting normal oxygen flow and breathing, and triggering breathlessness and coughing. Pulmonary fibrosis is one of these ILDs. 

Causes and Risk Factors

While the causes of pulmonary fibrosis vary, certain factors can increase an individual’s risk:

• Environmental and occupational exposures. Inhaling harmful pollutants, dust, or specific chemicals over a prolonged period can lead to PF.
• Radiation treatments. Some individuals who’ve had radiation therapy for chest tumors might develop PF as a side effect.
• Medications. Some drugs, including certain antibiotics, heart medications, and chemotherapy agents, have been linked to PF.
• Medical conditions. Diseases such as rheumatoid arthritis, lupus, and sarcoidosis can predispose an individual to PF.

What’s the difference between Pulmonary Fibrosis and Idiopathic Pulmonary Fibrosis?

The term “idiopathic” in medical jargon often denotes a condition whose cause is unknown. So, when we refer to idiopathic pulmonary fibrosis (IPF), we’re talking about a specific type of PF where the exact cause of the fibrosis remains unclear despite thorough investigation.

Here’s a more detailed breakdown:

• Cause. While PF can result from known causes (like those listed above), IPF has no identifiable cause.
• Progression and prognosis. IPF tends to have a more unpredictable progression and often a poorer prognosis compared to other forms of PF.
• Demographics. IPF is more commonly diagnosed in older adults, typically those above 60.
• Treatment. While both PF and IPF might be managed with similar strategies like oxygen therapy and pulmonary rehabilitation, certain medications specifically target IPF.

Understanding pulmonary fibrosis, its causes, and its idiopathic variant is essential for those affected, their families, and the broader medical community. Awareness can lead to early diagnosis, better management, and improved quality of life for patients. If you or a loved one have symptoms related to PF or IPF, it’s essential to consult with a healthcare professional. Knowledge is the first step toward effective care and management.