Slides
Page 1
©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Liquidia R&D Day Webcast presentation October 28, 2025
Page 2
2©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Forward-looking statements This presentation includes, and our response to questions may include, forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995 (“PSLRA”). All statements contained in this presentation other than statements of historical facts, including statements regarding our future results of operations and financial position, our strategic and financial initiatives, our business strategy and plans and our objectives for future operations, are forward looking statements. Such forward-looking statements, including statements regarding clinical trials, clinical studies and other clinical work (including the funding therefor, anticipated patient enrollment, safety data, study data, trial outcomes, timing or associated costs), regulatory applications and related submission contents and timelines, the timelines or outcomes related to patent litigation with United Therapeutics in the U.S. District Court for the District of Delaware and U.S. District Court for the Middle District of North Carolina, or other litigation between Liquidia and United Therapeutics or others, including rehearings or appeals of decisions in any such proceedings, the issuance of patents by the USPTO and our ability to execute on our strategic or financial initiatives, the potential for additional funding under the HCR Agreement, our anticipated use of net proceeds funded under the HCR Agreement, our estimates regarding future expenses, capital requirements and needs for additional financing, and potential revenue and profitability of YUTREPIA involve significant risks and uncertainties and actual results could differ materially from those expressed or implied herein. YUTREPIA’s approval and our launch of YUTREPIA remain subject to ongoing litigation in which United Therapeutics is seeking injunctive relief, which could block our ability to continue to sell YUTREPIA for one or both of PAH and PH-ILD. The words “anticipate,” “believe,” “continue,” “could,” “estimate,” “expect,” “intend,” “may,” “plan,” “potential,” “predict,” “project,” “should,” “target,” “would,” and similar expressions are intended to identify forward-looking statements. We have based these forward-looking statements largely on our current expectations and projections about future events and financial trends that we believe may affect our financial condition, results of operations, business strategy, short-term and long-term business operations and objectives and financial needs. These forward-looking statements are subject to a number of risks discussed in our filings with the U.S. Securities and Exchange Commission as well as a number of uncertainties and assumptions. Moreover, we operate in a very competitive and rapidly changing environment, and our industry has inherent risks. New risks emerge from time to time. It is not possible for our management to predict all risks, nor can we assess the impact of all factors on our business or the extent to which any factor, or combination of factors, may cause actual results to differ materially from those contained in any forward-looking statements we may make. In light of these risks, uncertainties and assumptions, the future events discussed in this presentation may not occur and actual results could differ materially and adversely from those anticipated or implied in the forward- looking statements. Although we believe that the expectations reflected in the forward-looking statements are reasonable, we cannot guarantee that future results, levels of activity, performance, achievements or events and circumstances reflected in the forward-looking statements will occur. We are under no duty to update any of these forward-looking statements after the date of this presentation to conform these statements to actual results or revised expectations, except as required by law. This presentation also contains estimates and other statistical data made by independent parties and by us relating to market size and growth and other data about our industry. This data involves a number of assumptions and limitations, and you are cautioned not to give undue weight to such estimates. In addition, projections, assumptions and estimates of our future performance and the future performance of the markets in which we operate are necessarily subject to a high degree of uncertainty and risk. This presentation includes long-term goals that are forward-looking, are subject to significant business, economic, regulatory and competitive uncertainties and contingencies, many of which are beyond our control and are based upon assumptions with respect to future decisions, which are subject to change. Actual results will vary, and those variations may be material. Nothing in this presentation should be regarded as a representation by any person that these goals will be achieved. We have no obligation under the PSLRA to update any forward-looking statements, and we undertake no duty to update our goals or to update or alter any forward-looking statements, whether as a result of new information, future events or otherwise.
Page 3
©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Liquidia Corporation is a biopharmaceutical company driven by science and compassion to revolutionize care for patients with challenging respiratory and vascular diseases through precise, innovative therapies that restore health and hope Improving drug delivery Using proprietary technologies Reducing burden of administration Helping patients breathe easier & live longer Enhancing drug delivery to the lungs to make every breath count
Page 4
4©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Ideal product profile for inhaled delivery Therapeutic goal is to optimize each aspect Targeted lung delivery Reduces off-target toxicity from oral, IV/SC delivery Tolerable Customizable and not dose limited Titratable Wide dose range to extend time on treatment Dosing frequency Easy and simple regimen Portable Convenience & ease-of-use to support compliance
Page 5
5©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Yutrepia addresses the first four elements now Therapeutic goal is to optimize each aspect Targeted lung delivery PRINT® Technology Tolerable INSPIRE (PAH) ASCENT (PH-ILD) Titratable Titratable in PAH, PH-ILD Dosing frequency 4x daily Portable Low-effort Trusted device YUTREPIA (treprostinil) inhalation powder
Page 6
6©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED L606 improves the inhaled product profile as the market expands Therapeutic goal is to optimize each aspect Targeted lung delivery Liposomal Technology Tolerable Further improved tolerability Titratable Over 48-weeks of treatment Dosing frequency 2x daily to minimize peak to trough excursions Portable Rapid, breath- actuated nebulizer L606 (liposomal treprostinil inhalation solution) YUTREPIA (treprostinil) inhalation powder
Page 7
7©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Meet today’s invited expert speakers Key opinion leaders Dr. Richard Channick, MD Saul Brandman Endowed Chair in Pulmonary Arterial Hypertension Co-Director, Pulmonary Vascular Disease Program Professor of Medicine Pulmonary and Critical Care Division David Geffen School of Medicine at UCLA Dr. Rajan Saggar, MD Professor of Medicine Director, Pulmonary Hypertension Program Co-Director Pulmonary Vascular Disease Program Lung & Heart-Lung Transplant and Pulmonary Hypertension Programs David Geffen School of Medicine, UCLA Dr. Ricardo Restrepo-Jaramillo Associate Professor of Medicine, Morsani College of Medicine Medical Director Center For Pulmonary Vascular Diseases University of South Florida/ Tampa General Hospital
Page 8
8©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Today’s outline Our objectives Understanding PAH and PH-ILD today ASCENT study in PH-ILD (Week 24 data) L606, treprostinil liposomal inhalation suspension in U.S. (Week 48 data) Physician Roundtable Q&A
Page 9
9©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Unmet Needs for Patients with PAH and PH-ILD Dr. Richard Channick, MD
Page 10
10©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED WHO clinical classification of PH CpcPH, combined post- and pre-capillary pulmonary hypertension; CTEPH, chronic thromboembolic pulmonary hypertension; ILD, interstitial lung disease; IpcPH, isolated post-capillary pulmonary hypertension; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension. 1. Humbert M et al. Eur Heart J. 2022;43(38):3618-3731. doi:10.1093/eurheartj/ehac237 2. Chang KY et al. J Am Heart Assoc. 2022;11(9):e024969. doi:10.1161/JAHA.121.024969 3. Kacprzak A et al. Diagnostics. 2023;13(14):2354. doi:10.3390/diagnostics13142354 Group 1 Group 2 Group 3 Group 4 Group 5 PAH • Idiopathic/heritable • Associated conditions PH associated with left heart disease • IpcPH • CpcPH PH associated with lung disease • Non-severe PH • Severe PH PH associated with pulmonary artery obstructions • CTEPH • Other pulmonary PH with unclear or multifactorial mechanisms • Hematological disorders • Systemic disorders Pressure in pulmonary arteries leads to vascular resistance, increased right ventricular pressure, right heart failure and death Chronic hypoxia can cause pulmonary vascular remodeling and subsequent elevation of pulmonary vascular resistance (PVR) Ultimately leading to the development of PH within the course of ILD3
Page 11
11©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED iNSIP up to 31% Prevalence estimates may change with increased focus on treatment Development of PH is a well-recognized complication of various ILDs1,7 IPF up to 86% at time of lung transplant ILD w/ SSc up to 31% ILD w/ CTD up to 21% IIPs2–4 Other2 CTD Associated5,6 Other lung conditions, including COPD1 CHP up to 44% CPFE up to 50% PH associated with lung disease1 Group 3 PAH1 Rare* Common* Group 1 ILD 48–55 cases per million adults1 *Within pulmonary hypertension. CHP, chronic hypersensitivity pneumonitis; COPD, chronic obstructive pulmonary disease; CPFE, combined pulmonary fibrosis and emphysema; CTD, connective tissue disease; IIP, idiopathic interstitial pneumonia; iNSIP, idiopathic non-specific interstitial pneumonia; IPF, idiopathic pulmonary fibrosis. 1. Humbert M et al. Eur Heart J. 2022;43(38):3618-3731. doi:10.1093/eurheartj/ehac237 2. Nikkho SM et al. Pulm Circ. 2022;12(3):e12127. doi:10.1002/pul2.12127 3. Parikh R et al. Pulm Circ. 2022;12(4):e12141. doi:10.1002/pul2.12141 4. Rahaghi FF et al. Chest. 2022;162(1):145–155. doi:10.1016/j.chest.2022.02.012 5. Young et al. Arthritis Rheumatol. 2017;71(8):1339–1349. doi:10.1002/art.40862 6. Hyldgaard et al. J Clin Med. 2021;10(21):4830. doi:10.3390/jcm10214830 7. Kacprzak A et al. Diagnostics. 2023;13(14):2354. doi:10.3390/diagnostics13142354
Page 12
12©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Recent real-world data reinforces poor outcomes in PAH & PH-ILD PVRI GoDeep Registry1 Yogeswaran et al “Hemodynamics and PDE5i Treatment in PH-ILD” Am J Respir Crit Care Med Vol 211, Iss 10, pp 1855–1866, Oct 2025 Risk of death is 2x higher in PH-ILD vs PAH PVR is the strongest predictor of survival ≤ 5 WU > 5 WU iPAH PAH PH-ILD 3 yrs 3 yrs
Page 13
13©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Symptoms can be similar with different diagnosis Early diagnosis and intervention may result in better clinical outcomes Dyspnea Syncope RV failureCough PH Fatigue ILD Monitor for signs and symptoms disproportionate to ILD severity3 • Altered heart sounds • Jugular venous distention • Signs of right heart failure • Ankle swelling/peripheral edema • Hepatomegaly/ascites • ILD requiring oxygen Perform screening tests 3 • Pulmonary function tests • CT scan • Oxygen saturation • 6MWD • Echocardiography Confirm PH diagnosis 3 • Right heart catheterization Abdominal distension Fatigue Right Heart Failure2 Weight gain Dyspnea PAH1 Syncope Edema Ascites Confirmation: Comprehensive PH Workup by PH Center1 Detection: Assessment by Lung and Heart Specialist1 • Echocardiography • CPET • 6MWD • Blood test • Echocardiography or cMRI • ABG or O2 saturation • Disease-specific HR-QoL • CPET & RHC PH 1. Humbert M, et al. Eur Heart J. 2022;43(38):3618-3731. doi:10.1093/eurheartj/ehac237 2. Watson RD, et al. BMJ. 2000;320(7229):236-239. doi:10.1136/bmj.320.7229.236; 3. Rahaghi FF et al. Chest. 2022;162(1):145–155. doi:10.1016/j.chest.2022.02.012
Page 14
14©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED CLD with PH mPAP > 20 mmHg PVR ≥3 WU mPAP >20 mmHg PAWP ≤15 mmHg PVR >2 WU mPAP >20 mmHg Recently updated 7th WSPH hemodynamic definitions BLPH=borderline pulmonary hypertension; CpcPH=combined post-capillary and pre-capillary pulmonary hypertension;CI=cardiac index; CLD=chronic lung disease; ERS=European Respiratory Society; ESC=European Society of Cardiology; IpcPH =Isolated post-capillary Pulmonary Hypertension mPAP=mean pulmonary arterial pressure; MPH=mild pulmonary hypertension; PAWP=pulmonary arterial wedge pressure; PVR =pulmonary vascular resistance; SPH=severe pulmonary hypertension; WSPH=World Symposium on Pulmonary Hypertension; WU=wood units. 1. Humbert M et al. Eur Heart J. 2022;43(38):3618–3731. doi:10.1093/eurheartj/ehac237 2. Simonneau G et al. Eur Respir J. 2019;53:1801913. doi:10.1183/13993003.01913-2018 3. Kovacs G, Bartolome S, Denton CP, et al. Definition, classification and diagnosis of pulmonary hypertension. Eur Respir J 2024; 64: 2401324 [DOI: 10.1183/13993003.01324-2024]. 4. Modified from Shlobin OA. Eur Respir J. 2024:2401200. 5. Piccari L, et al. Respiration. 2022;101(8):717-727. doi: 10.1159/000524263 PH-ILD1–4 Pre-capillary PHPH Right heart failure 5 4 6 2 1 0 0 1 2 3 4 5 6 6 5 4 6 2 1 10 20 30 40 50 60 mPAP (mmHg) PVR (Wood units) Mortality Hazard Ratio
Page 15
15©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Prostacyclin pathway will remain critical to treatment Targets the major pathologic changes that occur in PAH and PH-ILD Normal capillaries2 Capillaries in PH2 • Endothelial cell breaks • Impaired permeability • Protein loss Increased Hydrostatic Pressure H2O Normal Hydrostatic Pressure Alveolar Surface H2O H2O Patients with PAH have decreased levels of endogenous prostacyclin, a potent vasodilator and anti-inflammatory agent1 Prostacyclin mimetics have the potential to improve blood flow to the lungs, decrease PAP, improve exercise capacity, and/or improve QoL1 Smooth muscle proliferation13 Vasoconstriction11 Platelet aggregation1 2 Antiproliferative effects1 4
Page 16
16©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED The last 30 years has been an effort in optimizing delivery Inhaled has potential to maximize the balance of efficacy, tolerability and convenience Parenteral 1995 (epoprostenol) IV • Proved concept • Mortality benefit • Systemic toxicities, site pain Parenteral 2002 (treprostinil) SC 2004 (treprostinil) IV • Dose limiting systemic AEs • Infection • Injection site pain Oral 2013 (treprostinil) tid 2015 (selexipag) bid • Dose limiting systemic AEs • Requires up-titration, challenging • Time to titrate can be long Inhaled: Nebulized 2004 (iloprost) 6-9x 2009 (treprostinil) 4x • Large non-portable device • Prolonged nebulization time • Frequent administration • Limited dose range Inhaled: DPI 2022 (treprostinil) • Cough & Throat irritation • High resistance device • Limited dose range Inhaled: DPI 2025 (treprostinil) 1990s 2000s 2010s 2020s
Page 17
17©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Increasing interest to optimize inhaled delivery given the benefits Local delivery for local disease 1. Berkenfeld K, et al. AAPS PharmSciTech. 2015;16(3):479-490. doi:10.1208/s12249-015-0317 2. Roscigno RF, et al. Vascul Pharmacol. 2021;138:106840. doi:10.1016/j.vph.2021.106840 3. Hill NS, et al. Respir Care. 2015;60(6):794-805. doi:10.4187/respcare.03927 KEY FEATURES OF INHALED1-3 • Reduced risk of systemic adverse effects • Delivery of drug directly to lungs • Enhanced pulmonary specificity • Higher local drug conc. at lower dose • Improved ventilation/perfusion matching • May aid in patient compliance
Page 18
18©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Higher doses of inhaled treprostinil correlate to better outcomes *These data are from a Phase 4, retrospective, real-world analysis of patients prescribed inhaled treprostinil solution from a specialty pharmacy database between September 2009 and June 2018. Of the 6709 patients who met all study eligibility criteria, a random sample of 5000 patients was selected for further analysis using simple random sampling with equal probability. Shapiro S et al. Pulm Circ. 2021;11(4):20458940211052228. doi:10.1177/20458940211052228 of patients were titrated >9 breaths QID Retrospective analysis of 5,000 PAH patients (2009–18)* Only 28.5% Patients with 12 or more breaths had better clinical outcomes 6-minute walk distance (6MWD); 1. Nathan et al, CHEST Journal, February 2023, Vol. 163, Issue 2, P398-406; 2. Supplement to: Waxman et al, N Engl J Med 2021;384:325-34 Maximum study drug dose 4-6 breaths 7-9 breaths 10-12 breaths >12 breaths Inhaled tre (n) 6 37 77 1 Placebo (n) 2 24 92 2 -9.5 (-52.2, 33.1) 17.7 (-10.9, 46.2) 33.7 (15.8,51.7) -100 -50 0 50 100 Favors placebo Favors treprostinil Analyses of peak 6MWD at week 16 in PH-ILD1,2 LS mean difference (95% CI) 10 or more breaths of inhaled treprostinil had favorable improvements in 6MWD
Page 19
19©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Real-world challenges with Tyvaso DPI in PH-ILD Prostacyclin naïve patients showed worse tolerability and discontinued faster Rice et al, Tolerability and Efficacy of Treprostinil Dry-Powdered Inhaler in Patients with Pulmonary Hypertension Related to Fibrosing Interstitial Lung Disease at a Large Tertiary Referral Center, 2023 Pulmonary Hypertension Professional Network Symposium, September 28-30, 2023 [Poster] Tyvaso® and Tyvaso® DPI are registered trademarks of United Therapeutics Corporation Results Methods
Page 20
YUTREPIA is a dry-powder formulation of treprostinil enabled by PRINT® technology1 designed for Enhanced deep-lung delivery1–3 Ease of use with a low-effort device1,4–6 Titration to higher therapeutic doses1,7 PRINT® is a registered trademark of Liquidia Technologies, Inc. 1. Hill NS et al. Pulm Circ. 2022;12(3):e12119. doi:10.1002/pul2.12119 2. Garcia A et al. J Drug Deliv. 2012;2012:941243. doi:10.1155/2012/941243 3. Roscigno RF et al. Vascul Pharmacol. 2021;138:106840. doi:10.1016/j.vph.2021.106840 4. Patel S et al. Robustness of YUTREPIA, a dry-powder inhaled formulation of treprostinil, in patient misuse scenarios. Poster presented at: CHEST 2022 Annual Meeting; October 16-19, 2022; Nashville, TN. 5. Price D et al. Multidiscip Respir Med. 2015;10:36. doi:10.1186/s40248-015-0033-0 6. National Health Service Sunderland. Sunderland COPD Inhaler Guide. National Health Service; 2020. 7. YUTREPIA. Prescribing information. Liquidia Technologies, Inc; 2024. © 2025 Liquidia Technologies, Inc. All rights reserved.
Page 21
21©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Important related TEAEs decreased from Month 2 to Month 121,2, 3 Tyvaso transition patients 27% 9%8% 0% % of patients reporting AEs Patients naïve to prostacyclin 55% 21% 5% 0% % of patients reporting AEs INSPIRE study reinforced tolerability profile of Yutrepia over time 1. Yutrepia Prescribing Information, Table 2: Adverse Reactions Occurring in ≥ 4% of Patients in the INSPIRE Study; 2. Hill et al, “Safety and Tolerability of LIQ861 in Pulmonary Arterial Hypertension (PAH): Results From INSPIRE Study” Poster, ATS 2022; 3. Liquidia Data on File 100% reduction reported throat Irritation 72% reduction reported cough Mean dose increased 61% from Baseline to Month 12 Mean dose increased 338% from Baseline to Month 12 100% reduction reported throat Irritation 91% reduction reported cough Cough Throat irritation Baseline to Month 2 N=55 Month 2 to Month 12 N=53 Baseline to Month 2 N=55 Month 2 to Month 12 N=53 Cough Throat irritation Baseline to Month 2 N=66 Month 2 to Month 12 N=60 Baseline to Month 2 N=66 Month 2 to Month 12 N=60
Page 22
22©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Major themes of a changing landscape Standard of care is rapidly changing with new options Real world experience • Dose matters in PAH and PHILD • Inhaled therapy to avoid or delay the need for parenteral • Facilitating transition off parenteral with addition of sotatercept • Inhaled therapy allowing physicians to start prostacyclin earlier • New delivery methods are allowing higher doses of treprostinil • Transitioning from oral prostacyclin pathway drugs to inhaled
Page 23
23©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED ASCENT Study in PH-ILD @ Week 24 YUTREPIA (treprostinil) inhalation powder Dr. Rajan Saggar, MD
Page 24
24©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED 52-week TREATMENT PERIOD LIQ861+ RS00 Model 8 Dry Powder Inhalation (DPI) Device ASCENT Cohort A Open-label, Multicenter Study to Evaluate Safety and Tolerability of LIQ861 in Patients with Newly Diagnosed PH-ILD 6MWD=6-Minute Walk Distance; CE=cardiac effect; CT=computed tomography; CPFE=combined pulmonary fibrosis and emphysema; FEV1/FVC=forced expiratory volume in 1 second to forced vital capacity; ILD=interstitial lung disease; WU=wood units; PH-ILD=pulmonary hypertension associated with interstitial lung disease. Reference: LTI-401 Protocol, p.20-22. *Limited subset of patients ASCENT (NCT06129240) PATIENTS • N=54 • Age 18-80 years KEY INCLUSION CRITERIA • mPA ≥30 mmHg & PVR ≥3 WU or • mPA ≥21 mmHg & PVR ≥3 WU* • Baseline 6MWD ≥125m • CT Chest consistent with ILD or CPFE • FEV1/FVC ≥70% PRIMARY SAFETY ENDPOINTS • Incidence of treatment-emergent drug- or device-related AEs and serious AEs EXPLORATORY ENDPOINTS • Echocardiogram • Dyspnea-12, Emphasis 10, simplified cough score • 6MWD & cardiac effect • CT CHEST Screening 28 days Week 8 Week 16 Week 24Week Week 52D1 Baseline Target Dose (mcg) QID 132.5 159 185.526.5 EOS
Page 25
25©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Most PH-ILD patients remained on treatment at Week 24 ASCENT: Week 24 *Lung transplant listing was approved upon enrollment per agreement with Sponsor; transplantation was expected to occur ~ 3 months pending donor availability; Liquidia Data on File. Assessed for Eligibility (n=73) Received LIQ861 (n=54) Completed Week 24 of Study Assessment (n=39) Patients @ Week 8 @ Week 16 @ Week 24 Completed n (%) 53 (98.1) 43 (79.6) 39 (72.2) Missed Study visit - - 1 (1.9) - - Discontinued 1 (1.9) 10 (18.5) 15 (27.8) Physician Decision 1 (1.9) 1 (1.9) Withdrawal of Patient 2 (3.7) 2 (3.7) Protocol Violation 1 (1.9) 3 (5.6) Lung Transplant* 3 (5.6) 3 (5.6) Adverse Event 1 (1.9) 3 (5.6) 6 (11.1) • lung neoplasm • lung neoplasm • chronic pancreatitis • coronavirus • lung neoplasm (2) • bronchitis (1) • chronic pancreatitis (1) • coronavirus (1) • sudden cardiac death (1)
Page 26
26©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Baseline demographics ASCENT: Week 24 Naïve PH-ILD N=54 Age, mean ±SD, y 68.5 ± 8.9 Sex, Female (%) 28 ± 51.9% Duration of PH Diagnosis, y 0.5 ± 0.8 Duration of ILD Diagnosis, y 5.1 ± 5.7 ILD Subtypes (%) IIPs 26 (48.1%) Autoimmune ILDs 19 (35.2%) HP 1 (1.9%) Other ILDs 3 (5.6%) CPFE 5 (9.3%) # Background antifibrotics, n (%) Nintedanib 19 (35.2%) Pirfenidone 4 (7.4%) Background PH Drugs, n (%): PDE5i 7 (13%) Hemodynamics Mean ±SD mPAP (mmHg) 33.4 ± 8.4 PCWP (mmHg) 8.6 ± 3.3 Cardiac Output (L/min) 4.5 ± 0.9 PVR (WU) 6.0 ± 2.9 Pulmonary Function Test Mean ±SD FVC, L 2.07 ± 0.767 FVC (% predicted) 65.9 ± 20.7 FEV1/FVC 79.6 ± 17.1 DLCO (% predicted) 36.2 ± 13.9 Peak Inspiratory Flow Rate (L/min) Mean = 90.6 ± 22.3 Median = 90.0 Range = 39-120 Clinical Characteristic Mean ±SD 6MWD (Meters), ±SD 298.1 ± 80.3 NTPro-BNP (pg/ml) [GM=210.5] 611.0 ± 1246.0 Dyspnea-12 11.7 ± 6.8 EmPHasis-10 24.6 ± 9.7 Simplified Cough Score 1.3 ± 0.8
Page 27
27©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED At each time point, more than 80% patients receive ≥ 132.5 mcg ASCENT: Week 24 Liquidia Data on File; at Week 16, one patient discontinued dosing on clinic visit, which is recorded as 0 mcg 0.0 26.5 53.0 79.5 106.0 132.5 159.0 185.5 212.0 238.5 265.0 291.5 318.0 344.5 371.0 397.5 424.0 450.5 LIQ861 Dose (mcg) Week 8 n=51 Week 16 n=43 Comparable Breaths Per Session Equivalents of Tyvaso Week 24 n=39 median 49 46 43 40 36 33 30 27 24 21 18 15 12 9 6 3 0
Page 28
28©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED No significant changes in tolerability as dose was titrated Treatment-emergent adverse event.(TEAE); AE reported if > 5%; severe TEAEs (n=2): respiratory tract irritation (1); hypoxia (1) Liquidia Data on File Cumulative TEAEs Treatment-related TEAEs n (%) Week 8 Week 16 Week 24 Median Dose (mcg) 132.5 159 185.5 Cough 23 (42.6) 26 (48.1) 26 (48.1) Headache 7 (13.0) 10 (18.5) 10 (18.5) Oropharyngeal pain 3 (5.6) 4 (7.4) 4 (7.4) Fatigue 2 (3.7) 4 (7.4) 4 (7.4) Throat Irritation 2 (3.7) 4 (7.4) 4 (7.4) Diarrhea 2 (3.7) 3 (5.6) 3 (5.6) Dry Throat 3 (5.6) 3 (5.6) 3 (5.6) Cough Severity • Mild n=24 (44.4) • Moderate n=2 (3.7) • No treatment-related SAEs • Treatment related AE’s predominantly mild to moderate No discontinuations due to cough at Week 24 ASCENT: Week 24 (n=54)
Page 29
29©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Mean daytime cough scores remained stable as dose was titrated Wang Z, Wang M, Wen S, Yu L, Xu X. Types and applications of cough-related questionnaires. J Thorac Dis. 2019 Oct;11(10):4379-4388. Liquidia data on file Score Daytime Cough 0 No cough 1 Transient cough occasionally during the daytime 2 Frequent cough mildly affecting daily life 3 Frequent cough severely affecting daily life Instructions: The patient should circle the score that best describes their cough over the past two weeks 1.3 1.3 1.3 1.2 1.4 1.1 0 1 2 3 Baseline Week 8 Baseline Week 16 Baseline Week 24 Cough Scores from Baseline to Week 8, Week 16, Week 24 Mean Cough Score Week 8 n=51 Week 16 n=43 Week 24 n=39 ASCENT: Week 24
Page 30
30©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED 𝚫𝚫6MWD from baseline continued to increase ASCENT: Week 24 Mean ± Standard Deviation, Liquidia data on file +41 +31.5 +21.5 0 5 10 15 20 25 30 35 40 45 n=49Week 8 Week 16 Meters Week 24 n=41 n=37 Mean 24.3 ± 30.5 Mean 30.2 ± 36.1 Mean 39.6 ± 45.0 Median change from baseline in 6MWD
Page 31
31©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Most patients improved 𝚫𝚫6MWD by at least 40 meters (54%) ASCENT: Week 24 by patient Liquidia Data on File -100 -75 -50 -25 0 25 50 75 100 125 150∆ 6MW meters + ≥ 30m = 67.6% + ≥40m = 54.1% + ≥50m = 40.5%
Page 32
32©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Thoughts on PH-ILD at UCLA Summary of ASCENT at Week 24 • Patients continue to titrate to higher doses • No meaningful change in tolerability • Continued improvement in 𝚫𝚫6MWD to +41 meters
Page 33
33©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED L606 (treprostinil liposomal inhalation suspension) Dr. Rajeev Saggar
Page 34
34©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Illustrative example Classic formulation challenge to optimize exposure over 24 hours 0 24 hours
Page 35
35©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Pulses to form aerosol droplets Aerosol Droplets Single droplet contains liposomes Nanosized liposomes are delivered in micron sizes aerosol droplets Introduction to L606 Liposome technology Phospholipids Bilayer lipid membrane Treprostinil Inner Aqueous Compartment Aerosol Droplet ~4 μm contain liposomes A single liposome is between 100-140nmVibrating Mesh nebulizer
Page 36
36©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Liposome-containing aerosols are deposited in lung periphery Pulmonary pharmacokinetics of inhaled L606 liposomes Aerosols Liposome Alveoli
Page 37
37©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Liposomes extended drug release with salt-sensitive process ○ Minimal release rate in upper airway Controlled release rate in lower airway Simulated Lung Fluid Simulated Nasal Fluid UPPER AIRWAY • Low carbonic acid minimizes drug release in upper respiratory tract; trace amounts L606 quickly cleared by mucociliary action Low H2CO3 LOWER AIRWAY • Higher concentration of bicarbonate & carbonic acid in respiratory alveolar fluid help to control drug release H+ + Drug- Treprostinil Acid H2O + CO2 Drug- H+ + HCO- 2Stable H2CO3H+ + SALT-
Page 38
38©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED
Page 39
39©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Highly portable vibrating mesh nebulizer delivers L606 doses of less than 500µL Older technology FOX Mobile uses breath-activated technology L606 can be rapidly administered in about 1 minute L606 drug is supplied in 6 different dose strengths in disposable ampules that are used in combinations across a wide dose range Tyvaso® is registered trademark of United Therapeutics Corporation
Page 40
40©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Subjects were healthy volunteers 7.3-fold lower Cmax with similar AUC Lower cough incidence with L606 in SAD study Phase 1 cross-over study supports similar PK, better tolerability *Geometric mean (geometric CV%) 1. Phase 1 (Part B) as published in Tully et al. Clinical Pharmacokinetics of an Extended-Release Formulation of Inhaled Liposomal Treprostinil (L606) to Reduce Dosing Frequency [POSTER]. Pulmonary Vascular Research Institute (PVRI) 2024 Annual Congress; 2024 Feb 2, London. 66% 8% Cough Tyvaso (n=12) L606 (n=12) PK Parameters1 Tyvaso n = 12 L606 n = 12 Dose μg 54 51 Tmax h (median) 0.18 1.25 Cmax pg/mL 1090 (38.4) 140 (24.0) AUCinf h*pg/mL 1040 (27.5) 1050 (18.3) T ½ h 0.445 (14.6) 4.81 (29.2) CL/F L/h 52.0 (27.5) 48.8 (18.3)
Page 41
41©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Provides therapeutic levels during sleeping hours Modeled AUC of daily dosing (24hrs) is similar L606 offers more consistent exposure 0 400 800 1,200 1,600 2,000 2,400 2,800 0 – 12 hrs 12 – 24 hrs AUC (pg*hr/mL) Tyvaso 54 µg, qid L606 102 µg, bid L606 provides more consistent exposure over 24 hours with bid dosing Tully et al. Clinical Pharmacokinetics of an Extended-Release Formulation of Inhaled Liposomal Treprostinil (L606) to Reduce Dosing Frequency, Pulmonary Vascular Research Institute (PVRI) 2024 Annual Congress; 2024 Feb 2, London.
Page 42
42©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Clinical Data from U.S. Open-Label Study L606 (treprostinil liposomal inhalation suspension) Dr. Ricardo Restrepo-Jaramillo
Page 43
43©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED 48-week TREATMENT PERIOD L606 + Vibrating mesh nebulizer Phase 3 study evaluating safety & tolerability in PAH or PH-ILD patients Initiated by Pharmosa in 2021 and amended and led by Liquidia since July 2023 Link to clinicaltrials.gov: NCT04691154 PATIENTS • N=28 • Age 18-80 years KEY INCLUSION CRITERIA Cohort A (Transitions): • PAH and PH-ILD • Stable on Tyvaso Cohort B (Naïve): • PAH • Naïve to prostacyclin ● PRIMARY SAFETY ENDPOINTS • Incidence of treatment-emergent drug- or device-related AEs and serious AEs ∆ EXPLORATORY ENDPOINTS • Change in peak and trough 6MWD • Treatment Satisfaction Questionnaire for Medication (TSQM) transition patients only Screening 28 days Week 12 Week 24 Week 36Week Week 48Week 2,4,8 Endpoints ● ∆ ● ∆ ● ∆● ● ∆
Page 44
44©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Assessed for Eligibility (n=38) Received L606 (n=28) Most patients continued treatment with L606 Completed Week 48 of Study Assessment (n=24) Patients Completed Week 48 Visit, n (%) Completed 24 (85.7) Discontinued 4 (14.3) Protocol Violation 1 Adverse Event 3 • Chest discomfort (related, moderate) • Dyspnea (possibly related, mild) • Respiratory failure, transplant (not related, severe) L606 Open-Label (U.S.): Week 48
Page 45
45©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Participant demographics show naïve patients earlier in disease L606 Open-Label (U.S.): Week 48 *PH-ILD Patients on 2 PAH medicines included inhaled treprostinil & PDE5; body mass index (BMI) Cohort A Tyvaso Transition Cohort B PCY Naïve Overall PAH (N=18) PH-ILD (N=5) Total (N=23) PAH (N=5) (N=28) Age, years median (min, max) 64.5 (21, 75) 66 (56, 74) 65 (21, 75) 57 (29, 60) 61 (21, 75) Female, n (%) 15 (88.9) 1 (20.0) 16 (69.6) 5 (100) 21 (75.0) White, n (%) 15 (83.3) 4 (80.0) 19 (82.6) 3 (60.0) 22 (78.6) BMI, kg/m2 median (min, max) 27.3 (22.3, 39.7) 32.6 (27.3, 36.8) 28.3 (22.3, 39.7) 26.1 (21.7, 33.6) 27.4 (21.7, 39.7) Duration of Dx, yr median (min, max) 7.8 (0.6, 21.2) 3.9 (1.2, 9.9) 6.95 (0.6, 21.2) 1.05 (0.4, 15.5) 6.57 ( 0.4, 21.2) No. PAH Rx, n (%) 1 - 1 (20.0) 1 (4.3) 3 (60.0) 4 (14.3) 2 1 (5.6) 4 (80.0)* 5 (21.7) 2 (40.0) 7 (25.0) 3 17 (94.4) - 17 (73.9) - 17 (60.7) NYHA Class n (%) II 11 (61.1) 2 (40.0) 13 (56.5) 2 (50.0) 15 (55.6) III 7 (38.9) 3 (60.0) 10 (43.5) 2 (50.0) 12 (44.4)
Page 46
46©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Baseline disease characteristics show stable population of patients L606 Open-Label (U.S.): Week 48 *6MWD is comparable to other contemporaneous PAH studies at ~400m Six Minute Walk Distance (6MWD), N-terminal pro B-type natriuretic peptide (NT-proBNP), forced expiratory volume (FEV), forced vital capacity (FVC) Source: Liquidia Data on File Cohort A Tyvaso Transition Cohort B PCY Naïve Overall PAH (N=18) PH-ILD (N=5) Total (N=23) PAH (N=5) (N=28) 6MWD, meters* median (min, max) 390.5 (219.7, 525) 396 (231.7, 446) 396 (219.7, 525) 395 (240, 490) 395.5 (219.7, 525) NT-proBNP, pg/mL median (min, max) 176 (41, 1194) 152 (33, 452) 168 (33.3, 1194) 206.5 (45, 946) 168 (33, 1194)
Page 47
47©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Well tolerated with no treatment related SAEs or dose modifications L606 Open-Label (U.S.): Week 48 Six Minute Walk Distance (6MWD), forced vital capacity (FVC), forced expiratory volume (FEV), N-terminal pro B-type natriuretic peptide (NT-proBNP) Liquidia data on file Cohort A Tyvaso Transition Cohort B PCY Naïve Overall PAH (N=18) PH-ILD (N=5) Total (N=23) PAH (N=5) (N=28) Any TEAE 15 (83.3) 5 (100) 20 (87.0) 5 (100) 25 (89.3) Treatment Related TEAE 5 (33.3) 2 (40.0) 8 (34.8) 2 (40.0) 10 (35.7) Serious TEAE (SAE) 3 (16.7) 2 (40.0) 5 (21.7) 1 (20.0) 6 (21.4) Treatment Related SAE - - - - - Treatment related TEAE Led to Dose Reduction 1 (5.6) 1 (4.3) - 1 (3.6) Treatment Discontinuation 1 (5.6) 1 (20.0) 2 (8.7) 1 (20.0) 3 (10.7) Death - - - - -
Page 48
48©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED L606 Open-Label (U.S.): Week 48 Week 12 (n=26) Week 48 (n=24) Most patients titrated to doses comparable to >12 bps Tyvaso QID Breaths per session (bps) QID, Liquidia data on file 27% 42% 27% 4% Week 12 25+ bps 19-24 bps 13-18 bps 5-12 bps 8% 38% 25% 29% Week 48 25+ bps 19-24 bps 13-18 bps 5-12 bps L606 comparable dose equivalents to Tyvaso bps QID L606 Doses L606 comparable dose equivalents to Tyvaso bps QID 360mcg 42mcg Median 169 mcg L606 Doses 378mcg 42 mcg Median 229 mcg comparable to ~19 bps comparable to ~13 bps
Page 49
49©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED L606 is very tolerable with 4 (14%) patients reporting related cough L606 Open-Label (U.S.): Week 48 Liquidia data on file Most Common TEAEs Reported > 10 % TEAE Related TEAE % n % n Cough 32.1 9 14.3 4 Dyspnea 28.6 8 3.6 1 Fatigue 21.4 6 3.6 1 Dizziness 21.4 6 3.6 1 Nausea 10.7 3 3.6 1 Pruritis 10.7 3 3.6 1 COVID 19 17.9 5 - - Edema 14.3 4 - - Nasopharyngitis 10.7 3 - - Pneumonia 10.7 3 - - Upper Respiratory Tract Infection 10.7 3 - - NT-pro-BNP increased 10.7 3 - - Arthralgia 10.7 3 - - Back Pain 10.7 3 - - Hypotension 10.7 3 - - Cough Severity Related TEAE % n Mild 14.3 4 Moderate - - Leading to DC - -
Page 50
50©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED L606 Open-Label (U.S.): Week 48 All participants (N=24) Cohort B: PCY Naïve (N=4) Most patients maintained or improved 6MWD over time Mean ± Standard Deviation, Liquidia data on file +22.5 +17.2 0 10 20 30 40 Meters +22.5 +29 0 10 20 30 40 Meters Week 12 Week 48 Week 12 Week 48 Median change from baseline in 6MWD at peak Median change from baseline in 6MWD at peak Mean 23.6 ± 34.3 Mean 45.3 ± 55.0 Mean 72.3 ± 108.7 Mean 29.4 ± 64.4
Page 51
51©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED L606 Open-Label (U.S.): Week 48 All participants (N=24) Observed minimal variability between peak and trough measures Trough 6MWD conducted in morning of visit; Peak 6MWD conducted within 90-120 minutes after administering clinically observed dose Mean ± Standard Deviation, Liquidia data on file +22.5 +24.3 0 10 20 30 40 Meters Week 48, Trough Week 48, Peak Median change from baseline in 6MWD Mean 23.6 ± 57.8 Mean 29.4 ± 64.4
Page 52
52©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED -150 -100 -50 0 50 100 150 200 250 Most patients maintained or improved 𝚫𝚫6MWD at Week 48 L606 Open-Label (U.S.): Week 48 Liquidia Data on File; PAH patient (-118m) had knee surgery prior to conducting the 6MWD at Week 48 Liquidia data on file ∆ 6MW meters by patient at peak Any improvement = 80% + ≥30m = 30% PH-ILD PAH 3 of 4 pts ≥ 60m
Page 53
53©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Mean TSQM improved across all dimensions measured at 95% CI L606 Open-Label (U.S.): Week 48 Treatment Satisfaction Questionnaire for Medication (TSQM), Scores range from 0 to 100, with higher scores indicating greater satisfaction Liquidia data on file -20 0 20 40 Convenience Effectiveness Global Satisfaction Side Effects TSQM Score Mean Change (95% CI) from Baseline to Week 48/EOS Scores range from 0 to 100, with higher scores indicating greater satisfaction. Cohort A: Tyvaso Transitions
Page 54
54©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Thoughts on inhaled and sustained release Summary of L606 results • L606 was well-tolerated in patients over 48 weeks • Only 4 patients reports mild related cough over 48 weeks (14%) • Most patients maintained or improved in 6MWD • Observed minimal variability between peak and trough measures
Page 55
55©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Re-Spire Pivotal Study L606 (treprostinil liposomal inhalation suspension) Dr. Rajeev Saggar
Page 56
56©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED 56©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Seeking PAH & PH-ILD indication for L606 with one pivotal trial Three data sets required per FDA and EMA feedback 1. Phase 1 study NCT04041648; 2. Open label PAH & PH-ILD study NCT04691154 Comparable bioavailability to Tyvaso® in Phase 11Completed Open-label safety study in U.S. of PAH & PH-ILD2Ongoing Randomized placebo-controlled for efficacy in PH-ILDPlanned Powered by Bing Plan to initiate sites globally 20+ countries
Page 57
57©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED 344 patients 120 sites Phase 3, multi-center, randomized (1:1), double-blind, placebo-controlled, parallel group study Baseline (BL), Six Meter Walk Distance (6MWD), Time to Clinical Worsening (TTCW), Quality of Life (QOL), Clinical Worsening Event (CWE), Combined Pulmonary Fibrosis and Emphysema (CPFE) L606 b.i.d. Placebo b.i.d Blinded Treatment 24 weeks L606 b.i.d. Open Label Extension Phase (OLEP) Screening < 30 days Randomization 1:1 @ Baseline Primary Analysis 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 Exploratory Endpoint • Change from BL in hemodynamics (wk 24) • Change from BL to peak 6MWD (wk 8,12) • Change from Baseline in NT-proBNP • Change in Baseline in QOL endpoints Primary Endpoint • Change from BL in peak 6MWD (wk 16) Secondary Endpoint • Change from BL in peak 6MWD (wk 24) • Change from BL in trough 6MWD (wk 16) • TTCW from randomizationEarly Escape to OLEP for adjudicated CWE Stratification • Use of PDE5-I or Not (cap use at ~30%) • Baseline 6MWD (≤ 300 m or > 300 m) • PH-ILD etiology (CPFE or Other) 2 4 6 8 12 16 20 24 Q12 wk
Page 58
58©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Key enrollment eligibility criteria Any fibrotic ILD including Combined Pulmonary Fibrosis Emphysema ILD diagnosed on institutional HRCT Chest Eligibility based on central read from two independent thoracic radiologists Allow patients on approved anti-fibrotic treatment (pirfenidone/nintedanib) • HRCT Chest to be performed within12-months of screening • If HRCT Chest > 12-months a scan be performed during screening • Consistent with ILD – evidence of diffuse parenchymal disease • Evidence of “fibrosis” • Total Lung Emphysema must be ≤ 15% • Started at least 30-days prior to screening & stable dose with intent to continue throughout study • This could also include possible new drugs approved for IPF/fibrotic ILD during the study e.g., PDE4-inhibitor
Page 59
59©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Q&A Discussion
Page 60
60©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Dr. Richard Channick, MD Dr. Rajan Saggar, MD Dr. Roger Jeffs Chief Executive Officer Dr. Rajeev Saggar Chief Medical Officer Dr. Ricardo Restrepo- Jaramillo Q&A session
Page 61
61©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Portfolio built on optimizing inhaled delivery Drivers of future value for Liquidia’s programs Exposure drives Efficacy Tolerability drives Durability Convenience drives Compliance
Page 62
62©2025 LIQUIDIA CORPORATION ALL RIGHTS RESERVED Thank you for joining us today!