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Efficacy and Safety of Pirfenidone Treatment in HPS-ILD — PEARL  

Interstitial Lung Disease Clinical Trial

Official title:
Pirfenidone in the Treatment of Hermansky Pudlak Syndrome (HPS) - Related Interstitial Lung Disease (ILD)

Clinical Trial Summary

This research study will explore the safety and efficacy of the drug, pirfenidone, in patients with a diagnosis of Hermansky-Pudlak Syndrome (HPS) who have an associated interstitial lung disease (ILD) over a planned period of 56 weeks.

Clinical Trial Description

An open-label clinical study designed to evaluate the efficacy and safety of administering pirfeniodne for 52 weeks to subjects with HPS-ILD. Patients meeting the eligibility criteria without contraindications for the study will be provided pirfenidone 2403 mg/day. Efficacy will be evaluated through interval testing of pulmonary function tests, patient reported outcomes, adverse events and survival. Safety will be assessed by determining adverse events, hospitalizations, and all-cause mortality. ;

Study Design

Related Conditions & MeSH terms

Clinical Trial Details
NCT number NCT04193592
Study type Interventional
Source Thomas Jefferson University
Contact Tamra Perez, BSN
Phone 1-215-955-9181
Email tamra.perez@jefferson.edu
Status Not yet recruiting
Phase Phase 2
Start date December 1, 2019
Completion date December 31, 2022
View Details
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