Pulmonary Fibrosis: Causes, Diagnosis, and Management

Pulmonary fibrosis is a condition in which lung tissue becomes progressively scarred, reducing the lungs’ ability to transfer oxygen into the bloodstream. Symptoms often develop slowly and may include shortness of breath with exertion, a persistent dry cough, fatigue, and reduced exercise capacity. Because these signs overlap with other respiratory conditions, medical evaluation is important to determine whether scarring is present and to guide appropriate monitoring and care.

This article is for informational purposes only and should not be considered medical advice. Please consult a qualified healthcare professional for personalized guidance and treatment.

How the lungs are affected

The lungs rely on delicate air sacs (alveoli) and surrounding interstitial tissue to exchange oxygen and carbon dioxide. In pulmonary fibrosis, the interstitium becomes thickened with scar tissue, which stiffens the lungs and reduces their compliance. This change leads to lower lung volumes and impaired gas exchange, causing increased work of breathing and breathlessness. Over time, reduced oxygenation can affect other organs and may lead to secondary changes such as pulmonary hypertension. Regular lung function testing helps quantify how the lungs are functioning and whether the fibrotic process is stable or progressing.

Pulmonary fibrosis as a lung disease

Pulmonary fibrosis is a descriptive term for scarring patterns in the lung and represents one form of chronic lung disease. Idiopathic pulmonary fibrosis (IPF) is diagnosed when no clear cause is identified, but similar scarring can occur secondary to environmental exposures, medications, infections, or autoimmune disorders. Distinguishing between types of lung disease with fibrosis is important because prognosis and treatment options vary. Multidisciplinary assessment by pulmonologists, radiologists, and sometimes rheumatologists or pathologists improves diagnostic accuracy and informs individualized care plans.

Medical causes and risk factors

A variety of medical factors can contribute to pulmonary fibrosis. Known causes include occupational or environmental exposures (such as silica or asbestos), certain medications and chemotherapeutics, prior radiation to the chest, chronic infections, and connective tissue diseases like rheumatoid arthritis or systemic sclerosis. Age and a history of smoking are common associated factors, and some genetic variants increase susceptibility in families. Identifying and addressing modifiable contributors—such as stopping an offending medication or reducing exposure—can influence management and may slow progression in some cases.

Diagnosis and monitoring methods

Diagnosing pulmonary fibrosis typically combines clinical history, imaging, and physiological testing. High-resolution chest CT scans reveal patterns and distribution of scarring that suggest particular types of fibrosis. Pulmonary function tests, including spirometry and diffusion capacity measurements, quantify restriction and gas transfer impairment. Six-minute walk tests and arterial or pulse oximetry provide functional assessment and help monitor oxygen needs. In select cases, bronchoscopy or surgical lung biopsy may be needed for definitive tissue diagnosis. Ongoing monitoring at regular intervals helps track disease trajectory and response to medical interventions.