Pulmonary fibrosis

Pulmonary fibrosis is a condition in which the lungs become scarred over time. Symptoms include shortness of breath, a dry cough, feeling tired, weight loss, and nail clubbing. Complications may include pulmonary hypertension, respiratory failure, pneumothorax, and lung cancer.

Pulmonary fibrosis
Other namesInterstitial pulmonary fibrosis
Clubbing of the fingers in pulmonary fibrosis
SpecialtyPulmonology
SymptomsShortness of breath, dry cough, feeling tired, weight loss, nail clubbing
ComplicationsPulmonary hypertension, respiratory failure, pneumothorax, lung cancer
CausesTobacco smoking, environmental pollution, certain medications, connective tissue diseases, interstitial lung disease, unknown
TreatmentOxygen therapy, pulmonary rehabilitation, lung transplantation
MedicationPirfenidone, nintedanib
PrognosisPoor
Frequency>5 million people

Causes include environmental pollution, certain medications, connective tissue diseases, infections, and interstitial lung diseases. However, in most cases the cause is unknown, and termed idiopathic pulmonary fibrosis. Diagnosis may be based on symptoms, medical imaging, lung biopsy, and lung function tests.

No cure exists and only limited treatment options are available. Treatment is directed towards efforts to improve symptoms and may include oxygen therapy and pulmonary rehabilitation. Certain medications may be used to try to slow the worsening of scarring. Lung transplantation may occasionally be an option. At least 5 million people are affected globally. Life expectancy is generally less than five years.

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