Pulmonary Fibrosis: Causes, Diagnosis, and Care
Pulmonary fibrosis is a condition in which lung tissue becomes scarred and stiff, gradually reducing the lungs’ ability to move oxygen into the bloodstream. Early symptoms commonly include increasing shortness of breath with exertion, a persistent dry cough, and progressive reduction in exercise tolerance. Because these signs overlap with other respiratory conditions, timely medical evaluation that combines history, testing, and imaging helps clarify the diagnosis and guides management choices.
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.
What is pulmonary fibrosis?
Pulmonary fibrosis describes scarring and thickening of the interstitial tissue around the alveoli, the tiny air sacs where oxygen and carbon dioxide exchange occurs. This scarring reduces lung compliance and the effective surface area for gas transfer, producing a restrictive pattern on physiological testing. The term encompasses multiple patterns and causes; when no cause is found after comprehensive evaluation, clinicians may use the term idiopathic pulmonary fibrosis. Disease progression varies between individuals, so personalized assessment is important.
How does it affect the lungs?
Fibrotic changes in the lungs replace elastic tissue with collagen and other matrix components, narrowing small airways and thickening alveolar walls. Pulmonary function tests typically show reduced total lung capacity and a decreased diffusion capacity for carbon monoxide (DLCO), indicating impaired gas exchange. High-resolution CT imaging often demonstrates reticular changes, traction bronchiectasis, or honeycombing in advanced cases. These structural and functional changes explain symptoms such as exertional breathlessness and reduced exercise capacity, and they can lead to complications like pulmonary hypertension in some patients.
What causes this lung disease?
Causes of pulmonary fibrosis include environmental and occupational exposures (for example, silica or asbestos), connective tissue and autoimmune diseases (such as rheumatoid arthritis or systemic sclerosis), certain medications and chemotherapeutic agents, and prior thoracic radiation. Smoking and older age are associated with increased risk in some forms. When evaluation fails to reveal a clear trigger, the condition is classified as idiopathic pulmonary fibrosis. Genetic predisposition and altered repair processes in lung cells are areas of ongoing research and may inform evaluation in selected cases.
How is pulmonary fibrosis diagnosed medically?
Diagnosis integrates clinical history, physical examination, pulmonary function testing, and high-resolution CT imaging. Spirometry, lung volumes, and DLCO quantify the restrictive physiology and diffusion impairment. Laboratory testing can screen for autoimmune markers and systemic contributors. When noninvasive tests are inconclusive, bronchoalveolar lavage or surgical lung biopsy may provide tissue for histological analysis; these procedures are considered after multidisciplinary discussion because risks and benefits vary. A team approach including pulmonologists, radiologists, and pathologists improves diagnostic accuracy.
Treatment, monitoring, and health supports
Treatment aims to slow progression where evidence supports intervention, relieve symptoms, and maintain quality of life. Antifibrotic medications are indicated in certain forms and have been shown in clinical studies to reduce the rate of lung function decline for some patients; selection is medical and individualized. Supportive measures include supplemental oxygen for documented hypoxemia, pulmonary rehabilitation to improve exercise tolerance and daily function, immunizations to reduce infection risk, and symptom-directed therapies for cough and breathlessness. Regular monitoring with clinical assessment, pulmonary function tests, and imaging helps track disease course and guide adjustments in care.
Pulmonary fibrosis may have a variable trajectory; some people experience slow changes over years while others progress more quickly. Decisions about advanced therapies, including referral for lung transplant evaluation in eligible patients, are made based on disease severity, comorbidities, and patient goals. Coordination with primary care, respiratory specialists, and allied health services supports comprehensive medical and psychosocial care.
Pulmonary fibrosis is a complex lung disease with multiple causes, diagnostic steps, and management options. Ongoing follow-up, clear communication with healthcare providers, and attention to symptom changes support informed medical decision making and align care with individual health needs.
