An Extension Study of Subjects Who Received an Avalyn Inhaled Antifibrotic Agent (SAIL)

Inclusion Criteria:

• Previously participated in an Avalyn-sponsored inhaled antifibrotic clinical studyfor subjects with either idiopathic pulmonary fibrosis (IPF) (IPF subjects areexcluded from the US and Canada) or progressive pulmonary fibrosis (PPF) and withthe approval of the Study Physician.

Previous participation is defined as: Having completed the final visit of the Treatment Period on the full dose of study drug (either active or placebo).

• Male subjects and female subjects of childbearing potential (FOCBP) agree to usehighly effective contraception measures from the time of first dose of study drug (for the male subject) or the signing of the informed consent form (ICF) (for thefemale subject), during the study, and until 90 days after the last dose of studydrug. Subjects agree not to donate eggs or sperm during the same period.

Exclusion Criteria:

• Have not previously participated in an Avalyn-sponsored inhaled antifibrotic lead-instudy or if the subject was permanently discontinued from the lead-in study for anyreason. Subjects who discontinued study drug but continued to attend study visitsare ineligible.

• Subjects who experienced an exacerbation of asthma or of chronic obstructivepulmonary disease (COPD) requiring oral or systemic corticosteroids within 3 monthsof Day 1 (Screening/Baseline Visit).

• Subjects who experienced an acute exacerbation of IPF (IPF subjects are excludedfrom the US and Canada) or of PPF within 3 months of Day 1 (Screening/BaselineVisit).

• Participation in a concurrent clinical study or in a clinical study in which anyother investigational drug product aside from the Avalyn nebulized antifibroticmedication from their lead-in study was administered within the previous 30 days, or 5 half-lives of the previously administered investigational product, whichever isshorter. Subjects may be enrolled in registries.

• History of hypersensitivity and/or allergic reaction to pirfenidone or theexcipients to be used in this study.