Study of Inhaled Carbon Monoxide to Treat Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterized by
destruction of normal epithelial structure, proliferation of fibroblasts, and deposition of
connective-tissue matrix proteins. There are currently no effective therapies for IPF. Over
the past two decades, preclinical studies of inhaled low dose carbon monoxide (CO) have
shown that this biologically active diatomic gas possesses properties that would make it a
viable novel therapy for IPF. CO therapy has been well tolerated in Phase I and Phase II
human trials to date. This phase II study is designed to investigate whether IPF patients
show evidence of decreased peripheral blood levels of MMP7 and stability of secondary
indicators of disease progression after 3 months of inhaled therapy.

Inclusion Criteria:

- Adults above the age of 18 and equal to or below the age of 85

- Diagnosis of IPF by biopsy or

- ATS/ERS/ALAT Guidelines (Am J Respir Crit Care Med Vol 183. pp 788-824,2011)

- FVC greater than or equal to 50% predicted, greater than or equal to one month off
all medications prescribed for IPF

Exclusion Criteria:

- Evidence of active infection within the last month

- Significant obstructive respiratory defect

- Supplemental oxygen required to maintain an oxygen saturation over 88% at rest

- History of myocardial infarction within the last year, heart failure within the last
3 years or cardiac arrhythmia requiring drug therapy

- History of smoking within 4 weeks of screening

- Pregnancy or lactation

- Participation in another therapeutic clinical trial