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Oral Pirfenidone for the Pulmonary Fibrosis of HPS

Jan 6, 2023

This trial investigated the use of Pirfenidone, brand name Esbriet, to treat the pulmonary fibrosis of Hermansky-Pudlak Syndrome: Study Details

This original trial involved 21 participants and showed that the drug was somewhat helpful in slowing the development of pulmonary fibrosis.  It seemed to help most when given early in the development of fibrosis:  Study Details

The second study of Pirfenidone conducted by the National Institutes of Health was ended early due to a statistical issue in its construction.  It was not because the drug was, or was not found to be helpful.  It also offered further evidence of the safety of the drug and possible side effects:  Study Details