Pulmonary fibrosis | |
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Other names | Interstitial pulmonary fibrosis |
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Lung with end-stage pulmonary fibrosis at autopsy | |
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Clubbing of the fingers in pulmonary fibrosis | |
Specialty | Pulmonology |
Symptoms | Shortness of breath, dry cough, feeling tired, weight loss, nail clubbing[1] |
Complications | Pulmonary hypertension, respiratory failure, pneumothorax, lung cancer[2] |
Causes | Tobacco smoking, environmental pollution, certain medications, connective tissue diseases, interstitial lung disease, unknown[1][3] |
Treatment | Oxygen therapy, pulmonary rehabilitation, lung transplantation[4] |
Medication | Pirfenidone, nintedanib[4] |
Prognosis | Poor[3] |
Frequency | >5 million people[5] |
Pulmonary fibrosis is a condition in which the lungs become scarred over time.[1] Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing.[1] Complications may include pulmonary hypertension, respiratory failure, pneumothorax, and lung cancer.[2]
Causes include environmental pollution, certain medications, connective tissue diseases, infections, and interstitial lung diseases.[1][3][6] However, in most cases the cause is unknown, and termed idiopathic pulmonary fibrosis.[1][3] Diagnosis may be based on symptoms, medical imaging, lung biopsy, and lung function tests.[1]
No cure exists and only limited treatment options are available.[1] Treatment is directed towards efforts to improve symptoms and may include oxygen therapy and pulmonary rehabilitation.[1][4] Certain medications may be used to try to slow the worsening of scarring.[4] Lung transplantation may occasionally be an option.[3] At least 5 million people are affected globally.[5] Life expectancy is generally less than five years.[3]