Pulmonary fibrosis

Pulmonary fibrosis
Other namesInterstitial pulmonary fibrosis
Lung with end-stage pulmonary fibrosis at autopsy
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

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.

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

No cure exists and treatment options are limited. Treatment is directed toward improving symptoms and may include oxygen therapy and pulmonary rehabilitation. Certain medications may slow the scarring. Lung transplantation may be an option. At least 5 million people are affected globally. Life expectancy is generally less than five years.