An Open-Label Extension Study of Subjects who Received an Avalyn Inhaled Antifibrotic Agent

Prior treatment

• Have not previously participated in an Avalyn-sponsored inhaled antifibrotic lead-in study or if the subject was permanently discontinued from the lead-in study for any reason. Subjects who discontinued study drug but continued to attend study visits are ineligible.
• Participation in a concurrent clinical study or in a clinical study in which any other investigational drug product aside from the Avalyn nebulized antifibrotic medication from their lead-in study was administered within the previous 30 days, or 5 half-lives of the previously administered investigational product, whichever is longer. Subjects may be enrolled in registries.

Other illness

• Subjects who experienced an exacerbation of asthma or of chronic obstructive pulmonary disease (COPD) requiring oral or systemic corticosteroids within 3 months of Day 1 (Screening/Baseline Visit).
• History of non-adherence to medical regimens, unreliability, medical condition, mental instability or cognitive impairment that, in the opinion of the Investigator, could compromise the validity of informed consent, compromise the safety of the subject, or lead to non-adherence with the study protocol or inability to conduct the study procedures.
• History of hypersensitivity and/or allergic reaction to pirfenidone or the excipients to be used in this study.

Lab values

• Any conditions or abnormalities (including electrocardiogram [ECG] or laboratory abnormalities) which, in the opinion of the Investigator may compromise the safety of the subject or interfere with the subject participating in or completing the study.

Other

• Subjects who experienced an acute exacerbation of IPF or of PPF (as defined in Section 12.2.3) within 3 months of Day 1 (Screening/Baseline Visit).
• Is pregnant, nursing, or who plans to become pregnant while in the study.

• Provide written informed consent per the Institutional Review Board/Ethics Committee (IRB/EC)
• Previously participated in an Avalyn-sponsored inhaled antifibrotic clinical study for subjects with either IPF or PPF and with the approval of the Investigator. IPF is defined as: A specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the pathological pattern of usual interstitial pneumonia (American Thoracic Society 2000; Raghu et al, 2018).  PPF is defined as: At least 2 of 3 criteria (worsening symptoms, radiological progression, and physiological progression) occurring within the past year with no alternative explanation in a patient with an interstitial lung disease other than IPF (Raghu et al, 2022).  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) (defined as females who are fertile, following menarche and until becoming post-menopausal unless permanently sterile) agree to use highly 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 the female subject), during the study, and until 90 days after the last dose of study drug. Subjects agree not to donate eggs or sperm during the same period. Male subjects must use a condom and female partners of male subjects who are of childbearing potential must use a highly effective method of contraception, defined below: a. A highly effective method of contraception is one that results in a low failure rate (i.e., <1% per year) when used consistently and correctly. The acceptable methods of contraception include: i. sexual abstinence, defined as refraining from heterosexual intercourse during the entire period of risk associated with the study treatments. The reliability of sexual abstinence needs to be evaluated in relation to the duration of the clinical study and the preferred and usual lifestyle of the subject. ii. a vasectomized partner iii. bilateral tubal occlusion iv. any effective intrauterine device/hormone-releasing system, and progesterone-only (oral, injectable, or implantable) or combined (estrogen- and progesterone-containing; oral, intravaginal, or transdermal) hormonal contraception associated with inhibition of ovulation. Note: Periodic abstinence (calendar, symptothermal, post-ovulation methods), withdrawal (coitus interruptus), spermicides only, and lactational amenorrhea method are not acceptable methods of contraception. b. The efficacy of oral hormonal contraceptives may be compromised by vomiting and/or diarrhea or other conditions where the drug absorption may be reduced. Advise women taking oral hormonal contraceptives experiencing these conditions to use alternative highly effective contraception. Note: Permanent sterilization methods include hysterectomy, bilateral salpingectomy, and/or bilateral oophorectomy. A postmenopausal state is defined as no menses for 12 months without an alternative medical cause. In the absence of 12 months of amenorrhea, a single folliclestimulating hormone measurement is insufficient to document menopause.
• Willingness to comply with all study visits and requirements.
• Male or female at least 18 years of age at Screening/Baseline (Day 1).