Study Background

This pilot study will determine safety and efficacy of a hyaluronan synthesis inhibitor, hymecromone/H01/4-methylumbelliferone in decreasing hyaluronan levels in serum and sputum of patients with progressive interstitial lung disease (ILD).

Progressive interstitial lung disease can be fatal, and few therapeutic options exist. Drugs that can arrest or reverse the progression of fibrosis are urgently needed. The extracellular matrix component hyaluronan is enriched in fibrotic tissue and plays a mechanistic role in the progression of lung fibrosis and its sequelae. We hypothesize, that inhibition of hyaluronan expression will be beneficial in lung fibrosis. 

Participants will be given two 400 mg oral H01 two times a day (total dose: 1600 mg/day). Participants will be on treatment continuously for 12 weeks. During the treatment period, participants will be monitored according to the schedule of assessments. Participant final follow up will take place 4 weeks after the end of treatment. 

Primary Objective

Evaluate the efficacy of H01 in reducing hyaluronan levels in participants with progressive interstitial lung disease. 

Secondary Objectives

1. Evaluate the safety and tolerability of oral H01 in participants with progressive interstitial lung disease.
2. Evaluate the change in clinical and functional measures in participants with progressive interstitial lung disease treated with H01.
3. Evaluate biomarkers of fibrosis in participants with progressive interstitial lung disease treated with H01.
4. Evaluate pharmacokinetic changes from baseline in participants with progressive interstitial lung disease treated with H01.