Unilateral Pulmonary Fibrosis Due to Absence of Right Pulmonary Artery

Pulmonary fibrosis is typically a bilateral, progressive interstitial lung disease that is often idiopathic but can be associated with risk factors such as advanced age, environmental exposure, and drug toxicity. The pathophysiology is incompletely understood but involves transforming growth factor. The treatment choices for idiopathic disease include medical therapy that manipulates epigenetic pathways and lung transplantation. Here we present a 30-year-old female with no identifiable risk factors who developed unilateral pulmonary fibrosis. Clinical investigation eventually revealed a congenitally absent right pulmonary artery which was presumed to cause her unilateral disease. In contrast to idiopathic pulmonary fibrosis, treatment options for pulmonary fibrosis due to unilateral absence of a pulmonary artery include ipsilateral pulmonary vasculature embolization and/or pneumonectomy if disease is unmanageable without therapy.