Inhaled NAC in Treatment of IPF

This study plans to learn more about the safety and tolerability of inhaled N-Acetylcysteine
(NAC) in patients with pulmonary fibrosis. The study will also create a bank of data, blood,
and sputum from IPF patients for future research.

NAC is a medication used to loosen thick mucus. NAC was initially licensed for use in 1968.
It is on the World Health Organization's List of Essential Medicines, the most effective and
safe medicines needed in a health system, and it is available as a generic medication and is
not very expensive. Inhaled NAC has been used as a mucus-dissolving therapy in respiratory
conditions with excessive and/or thick mucus production.

Inclusion Criteria:

- Diagnosed by study team with expertise in IPF utilizing standard ATS/ERS definition of
"probable" or "definite" IPF

- DLCO >50% predicted

- FVC >60% predicted

- FEV1/FVC > 0.7

Exclusion Criteria:

- History of bronchospasm (requiring treatment)

- Current acute exacerbation of their IPF disease

- Current smoker

- Supplemental O2 requirement > 4 liters/min via nasal cannula

- History of asthma, COPD, coronary artery disease, or cancer

- Currently using NAC, hypertonic saline, or DNase (dornase alfa) inhalation therapy