PIRFENIDONE FOR TREATMENT OF PULMONARY FIBROSIS IN HERMANSKY-PUDLAK SYNDROME-1

PURPOSE: Progressive pulmonary fibrosis is a leading cause of death in individuals with Hermansky-Pudlak syndrome (HPS)-1. Treatment for pulmonary fibrosis in HPS-1 is limited, but one potential drug is pirfenidone. The purpose of this study is to retrospectively review medical records, pulmonary function tests (PFTs), and high-resolution computed tomography (HRCT) scan findings of three subjects who have been treated long-term with...