IRulya Therapeutics (QNCX) investor relations material

Pipeline and milestones

• Once-daily inhaled rapamycin LAM-001 is in Phase 2 development for PH-ILD, BOS and SAPH; estimated US/EU populations are ~200K, ~30K and ~60K, respectively.

• PH-ILD Phase 2b was expected to start mid-2026, with data anticipated 1Q28; BOS Phase 2 data are expected 1Q27 and SAPH Phase 2 data 4Q28.

• US/EU orphan disease status has been granted for multiple indications; FDA feedback from end-of-Phase 2 and pre-IND meetings was supportive.

PH-ILD clinical program

• In the Phase 2a evaluable population (N=6), 24-week changes included +81.3 m in 6MWD, +5.4% in predicted VO2 max, -25.5% in exercise PVR and -28.1% in supine PVR.

• In the PH-ILD subgroup (N=4), 24-week changes included +67.4 m in 6MWD, +6.7% in predicted VO2 max, -35.3% in exercise PVR and -33.9% in supine PVR.

• All 10 patients were WHO Functional Class III at baseline; 6 improved to Class II by Week 12, all completers reached Class II by Week 24, and 2 patients improved to Class I.

• Through 24 weeks, reported drug-related AEs were cough in 2 patients and gingivitis in 1; no AE-related dose interruptions, drug-related discontinuations or drug-related serious AEs.

• Phase 2b plans 75 patients randomized 1:1:1 to 100 μg, 200 μg or placebo for 24 weeks, followed by a 12-month open-label extension; primary endpoint is change in PVR.

BOS and SAPH programs

• BOS Phase 2 enrollment completed in January 2026; an interim safety review in February found 5 of 19 patients with FEV1 decline >10% while the study remained blinded; data are anticipated 1Q27.

• Retrospective BOS data cited showed improved or stabilized FEV1 in 8 of 11 patients (73%) after oral rapamycin treatment.

• BOS is described as the leading cause of death after lung transplantation; the presentation cites ~17K US and ~11K EU post-transplant patients and no FDA-approved drugs.

• SAPH Phase 2 was expected to initiate in 4Q26, supported by proof-of-concept clinical efficacy data from a completed PH study.