Slide 1: Investor Presentation
Investor Presentation August 2026 We’re in. For patients. 1
Slide 2: Forward Looking Statements
Forward Looking Statements The forward-looking statements in this presentation are based upon the Company’s current expectations and beliefs, with MAC lung disease, among other things; development of unexpected safety or efficacy concerns related to our and involve known and unknown risks, uncertain
Slide 3: Additional Disclaimers
Additional Disclaimers Please be aware that TPIP, INS1201, INS1202, INS1148, and INS1033 are investigational products that have not been approved for sale or found safe or effective by the FDA or any regulatory authority. In addition, ARIKAYCE has not been approved for the treatment of all patients
Slide 4: Three Therapeutic Areas, One Goal: Develop First- and Best-in-Class Therapies
Three Therapeutic Areas, One Goal: Develop First- and Best-in-Class* Therapies Immunology Neuro & Respiratory & Inflammation Other Rare Research & Business Development * “Best-in-disease/best-in-class" indicates a profile that could be considered more attractive than other treatment options for a pa
Slide 5: First-in-Class and Potentially Best-in-Class Assets Across Each Phase of Development
First-in-Class and Potentially Best-in-Class* Assets Across Each Phase of Development Pre-Clinical Phase 1 Phase 2 Phase 3 Commercial BRINSUPRI® (Brensocatib)**: Non-Cystic Fibrosis Bronchiectasis (NCFB) ARIKAYCE® (Amikacin Liposome Inhalation Suspension): Refractory MAC Lung Disease Amikacin Liposo
Slide 6: Second-Quarter Performance Supports Updated Full-Year Guidance for BRINSUPRI
Second-Quarter Performance Supports Updated Full-Year Guidance for BRINSUPRI Full-Year 2026 Revenue Gross-to-Net GUIDANCE GUIDANCE $1.25B to Mid-20%s to $1.4B† High-20%s Previously Previously “Greater than $1B” “Mid-20%s to Low-30%s” $450M to Low-20%s to $470M‡ Mid-20%s Unchanged Unchanged B: billio
Slide 7: Peak Revenue Estimate Raised to $14B+ for Three Lead Assets
Peak Revenue Estimate Raised to $14B+ for Three Lead Assets Across Their Respective Indications* Prior Updated $5B+ $7B+ Total Peak Bronchiectasis Bronchiectasis† Revenue Estimate* TPIP (Investigational Product) $2B+ PAH & PH-ILD Only $6B+ PAH, PH-ILD, PPF, IPF $14B+ $1B+ $1B+ All MACLD All MACLD B:
Slide 8: Respiratory
Respiratory THERAPEUTIC AREA 8
Slide 9: BRINSUPRI (brensocatib)
Brinsupri° (brensocatib)
Slide 10: BRINSUPRI Approved by the FDA
BRINSUPRI Approved by the FDA • Approved in 10 mg and 25 mg tablet form for adults and adolescents 12+ years with NCFB • Indication label has no requirement on number of documented pulmonary exacerbations • 25 mg dose label includes details on statistically significant benefits on FEV1 • Label refle
Slide 11: Strong Second-Quarter Revenue Results Increase Confidence to Raise Full-Year 2026 Guidance
Strong Second-Quarter Revenue Results Increase Confidence to Raise Full-Year 2026 Guidance Q2 2026 RESULTS GUIDANCE $1.25 $ 309.2M $1.4B to Global Net Revenues† Full-Year 2026 Revenues M: million | B: billion | Q: quarter | † Unaudited revenues for three months ended June 30, 2026 11
Slide 12: Third Full-Quarter Revenues Continue to Outperform Analogs
Third Full-Quarter Revenues Continue to Outperform Analogs, Enabled by Operational Excellence BRINSUPRI ® WINREVAIR OHTUVAYRE DUPIXENT ® FASENRA® REZDIFFRA TEZSPIRE 1 2 3 4 5 6 7 350 309 3rd full quarter BRINSUPRI revenues exceeded 4th full 300 280 quarter revenues of analog launches‡ 250 208 200 20
Slide 13: Strategic Collaborations and Future Studies Aim to Expand Long-Term Evidence
Strategic Collaborations and Future Studies Aim to Expand Long-Term Evidence European Multi-centre Bronchiectasis Audit and Research Collaboration (EMBARC) 3-year open-label study Ongoing plans to Up to 3,000 patients demonstrate long-term evidence through Across six countries Phase 4 and v real-wor
Slide 14: Brensocatib in Bronchiectasis Opportunity Could Potentially Reach >1M Patients
Brensocatib in Bronchiectasis Opportunity Could Potentially Reach >1M Patients* U.S. EU5† Japan TAM TAM TAM Diagnosed with Bronchiectasis1 500K 600K 150K Undiagnosed‡ Bronchiectasis2 2.4M 2.1M 0.8M Asthma or COPD3 32M 27M 17M TAM: total addressable market | U.S.: United States | *Pending regulatory
Slide 15: ARIKAYCE
a eete A ARIKAYCE’ (amikacin liposome inhalation suspension) 15
Slide 16: Executing Commercially While Preparing for Label Expansion in the U.S. & Japan
Executing Commercially While Preparing for Label Expansion in the U.S. & Japan* Q2 2026 Revenue† Preparing for Potential Label Expansion ✓ Filed sNDA with FDA in July in newly diagnosed MACLD patients ► U.S. label expansion would extend ARIKAYCE treatment to all MACLD patients, if approved U.S. Worl
Slide 17: ARIKAYCE Has Potential to Be the Best-in-MACLD Treatment
ARIKAYCE Has Potential to Be the Best-in-MACLD Treatment U.S. EU5† Japan TAM TAM TAM Refractory MAC4 12-17K 1.4K 15-18K MACLD4 95-115K 14K 125-145K We anticipate marketing ARIKAYCE in newly diagnosed MACLD patients at same price and dosage as currently available ARIKAYCE, if approved* U.S.: United S
Slide 18: ARIKAYCE Phase 3b in MACLD: ENCORE
ARIKAYCE Phase 3b in MACLD: ENCORE Primary Endpoint One Month Three Months • Change from baseline in Respiratory Symptom Score Off Treatment Off Treatment (RSS)3 at Month 13 (one month off treatment) Months 1-12 Month 13 Month 15 Multiplicity-Controlled Secondary Endpoints* 425 adults with new occur
Slide 19: Results Show Early Treatment With ARIKAYCE + Multidrug Therapy Can Improve Outcomes
Results Show That Early Treatment With ARIKAYCE + Multidrug Therapy Can Significantly Improve Outcomes for MACLD Patients Treatment with ARIKAYCE1 vs. Comparator2 showed… RSS Culture Safety & Improvement Conversion Tolerability ✓ Statistically significant at Month 13* ✓ Statistically significant ✓ >
Slide 20: Treprostinil Palmitil Inhalation Powder (TPIP)
Treprostinil Palmitil Inhalation Powder (TPIP) TPIP: treprostinil palmitil inhalation powder | FDA: Food and Drug Administration | Note: TPIP is an investigational product that has not been approved for sale or found safe or effective by the FDA or any regulatory authority 20
Slide 21: Investigational Once-Daily Prostanoid With Potential for Best-in-Class Efficacy
Investigational Once-Daily Prostanoid With the Potential to Deliver Best-in-Class† Efficacy With Enhanced Tolerability Potential Advantages of TPIP Efficacy* Safety* Convenience** • Inhaled therapy delivered • Potential for reduced systemic • Administered once daily vs. directly to the lung tissue s
Slide 22: Four TPIP Indications Represent Substantial Commercial Opportunities
Four TPIP Indications Represent Substantial ~$300K Commercial Opportunities U.S. Pricing TYVASO DPI® Benchmark List Price‡ U.S. EU5† Japan TPIP TAM TAM TAM PAH5 35K 40K 15K PH-ILD6 50K 65K 20K PPF7 145K 90K 20K IPF8 125K 75K 20K TPIP: treprostinil palmitil inhalation powder | TAM: total addressable
Slide 23: Expansive Registrational Program Across Four Indications Progressing on Track
Expansive Registrational Program Across Four Indications Progressing on Track PH-ILD PAH • TPIP's broad dosing range could provide PALM-ILD study PALM-PAH study actively enrolling actively enrolling physicians with the flexibility to optimize the balance between efficacy and tolerability TPIP • The
Slide 24: TPIP Phase 3 in PH-ILD: PALM-ILD
TPIP Phase 3 in PH-ILD: PALM-ILD NCT07179380 Trial summary PALM-ILD (Week 24) Primary Endpoint • Change in baseline exercise capacity (6MWD) Treatment period at peak exposure (1-3 hours post-dose) 30 days 4 weeks 24 weeks 4-week initial Secondary Endpoints Dose increases titration to the allowed up
Slide 25: TPIP Phase 3 in PAH: PALM-PAH
TPIP Phase 3 in PAH: PALM-PAH NCT07481981 Trial summary PALM-PAH (Week 24) Primary Endpoint • Change in baseline exercise capacity (6MWD) Treatment period at peak exposure (1-3 hours post-dose) 30 days 4 weeks 24 weeks Secondary Endpoints 4-week initial Dose increases titration to the Steady-state •
Slide 26: Positive OLE Study Results in PAH Reinforce TPIP's Potential
Positive OLE Study Results in PAH Reinforce TPIP’s Potential to Become the Prostanoid of Choice 12-Month Data from Phase 2b Study Results Ongoing OLE Study‡ 55 † PVR Reduction1 ~ 35% * 6MWD Improvement4 (meters) + 60% † NT-proBNP Reduction5 ~ 35.5 s 6MWD Improvement2 ^ (meters) + ~80% WHO FC I or II
Slide 27: FDA Grants Treprostinil Palmitil Orphan Drug Designation in PAH
FDA Grants Treprostinil Palmitil Orphan Drug Designation in PAH Major Contribution Efficacy to Patient Care Placebo-Adjusted Improvement in 6MWD † at Week 161 TPIP Profile 35.5 * Potential + Clinical Once-daily therapy with: Superiority ✓ continuity of parenteral meters treatment ✓ localization of i
Slide 28: TPIP's Profile in the Evolving PAH Treatment Landscape
TPIP’s Profile Could Represent a Compelling Option in the Evolving PAH Treatment Landscape TPIP Investigational product that has not been approved for sale or found safe or effective by the FDA or any regulatory authority OLE (SOTERIA) RCT (16 weeks) OLE (12 months) RCT (12 weeks) OLE (12 months) RC
Slide 29: INS1148
00 t re im n Z — 29
Slide 30: INS1148: Potential First-in-Class Therapy for Respiratory and I&I Diseases
INS1148: Potential First-in-Class Therapy for Respiratory and I&I Diseases SCF248/c-Kit Signaling Pathway Inflammatory Mediators INS1148* is designed to selectively target SCF248, Immune Cells with SCF248 c-Kit Receptors a specific isoform of SCF (c-Kit receptor ligand1) (Mast Cells, ILC2, Eosinophi
Slide 31: Respiratory Therapeutic Area Portfolio & Pipeline
Respiratory Therapeutic Area Portfolio & Pipeline Pre-Clinical Ph1 Ph2 Ph 3 Commercial BRINSUPRI® (brensocatib) Bronchiectasis* Anticipated Catalysts ARIKAYCE® Refractory MAC MAC Lung Disease 2H:26 | ARIKAYCE: Review ENCORE data with PMDA TPIP PH-ILD 2H:26 | PALM-PPF: Ph3 trial initiation PAH PPF 1H
Slide 32: Immunology & Inflammation
Immunology & Inflammation THERAPEUTIC AREA 32
Slide 33: Immunology & Inflammation Therapeutic Area Pipeline
Immunology & Inflammation Therapeutic Area Pipeline Pre-Clinical Ph1 Ph2 Ph 3 Commercial Anticipated Catalysts DPP1 Inhibitors INS1033: RA INS1033: UC Q3:26 | RA: Ph1 study initiation in healthy volunteers RA: rheumatoid arthritis | UC: ulcerative colitis | Q: quarter | Ph: phase | Initiation indica
Slide 34: Neuro & Other Rare
Neuro & Other Rare THERAPEUTIC AREA 34
Slide 35: INS1201 Phase 1 in DMD: ASCEND
INS1201 Phase 1 in DMD: ASCEND NCT06817382 Trial summary ASCEND Key Trial Information Primary Endpoint • Patients must be ambulatory at time of screening • Safety and tolerability (up to Week 96) • Single dose via intrathecal administration Secondary Endpoints Cohort 2 • Muscle dystrophin levels (DN
Slide 36: INS1202 Phase 1 in ALS: ARMOR
INS1202 Phase 1 in ALS: ARMOR NCT07290062 Trial summary ARMOR Key Trial Information • Cohort enrollment will be staggered, with a 30-day safety observation between the first 3 participants in a Primary Endpoint cohort regardless of ALS subpopulation • Safety and tolerability (up to Week 48) • ALS su
Slide 37: Neuro & Other Rare Therapeutic Area Pipeline
Neuro & Other Rare Therapeutic Area Pipeline Pre-Clinical Ph1 Ph2 Ph 3 Commercial Anticipated Catalysts Gene Therapy ’26/’27 | ASCEND DMD updates INS1201: DMD INS1202: ALS* INS1203: Stargardt ’26/’27 | ARMOR ALS updates 2027 | IND for Stargardt filing DMD: Duchenne muscular dystrophy | ALS: amyotrop
Slide 38: Research Engine
Research Engine PRE-CLINICAL PROGRAMS & BUSINESS DEVELOPMENT 38
Slide 39: Multi-Dimensional Early-Stage Research Portfolio
Multi-Dimensional Early-Stage Research Portfolio 30 Synthetic Rescue Number of Active > Pre-Clinical Programs De-Immunized Therapeutic Proteins % of Overall Spend on Pre-Clinical Programs < 20% Gene Therapy Estimated Number of INDs Filed Per Year 1-2 Other Research IND: investigational new drug appl
Slide 40: Pre-Clinical Research Programs and Business Development Expected to Fuel Future Waves of Growth
Pre-Clinical Research Programs and Business Development Expected to Fuel Future Waves of Growth Our Approach Our Pre-Clinical Platforms Low upfront costs to acquire technologies Intrathecally Delivered Gene Therapy End-Joining Other Expenditures Gene Therapy <20% Pre-clinical Deimmunized Protein Pro
Slide 41: Our Culture Is Our Greatest Strength
Our Culture Is Our Greatest Strength In a recent survey* Accolades that span the globe >90% of employees who responded said they felt: Proud to work at Insmed Inspired by what we do Confident in Insmed’s future Driven to do their best work * The 2025 annual Insmed Pulse Survey included 92% participa
Slide 42: Appendix
Appendix 42
Slide 43: Strong, Long-Dated Patent Exclusivity
Strong, Long-Dated Patent Exclusivity European U.S. Japan Union Current Potential Current Potential Current Potential Exclusivity Exclusivity Exclusivity Exclusivity Exclusivity Exclusivity ARIKAYCE 2035 2041a 2035 2041a 2035 2041a BRINSUPRI 2040 2040+b 2039 2040+c 2035 2040c TPIP 2034 2041d 2034 20
Slide 44: Manufacturing Country of Origin
Manufacturing Country of Origin ARIKAYCE Brensocatib TPIP API API API China1 Spain Taiwan Drug Product Drug Product Drug Product Canada UK Canada U.S. To come online Project underway to establish brensocatib secondary source manufacturing in the U.S. API: active pharmaceutical ingredient | U.S.: Uni
Slide 45: Brensocatib Phase 3 in Bronchiectasis: ASPEN
RESPIRATORY | Brensocatib (Bronchiectasis) Brensocatib Phase 3 in Bronchiectasis: NCT04594369 Trial summary Treatment period 6 weeks 52 weeks 4 weeks ASPEN Primary Endpoint Brensocatib 10 mg once daily • Annualized rate of adjudicated pulmonary exacerbations* over 52 weeks 1,721 patients randomized
Slide 46: Phase 3 ASPEN Study Primary Endpoint Achieved Statistical Significance on Both Doses
RESPIRATORY | Brensocatib (Bronchiectasis) Phase 3 ASPEN Study a Clear Win: Primary Endpoint Achieved Statistical Significance on Both Doses Brensocatib 10 mg Brensocatib 25 mg compared to placebo compared to placebo Primary Endpoint Reduction in annualized rate of PEs 21.1% p = 0.0019* 19.4% p = 0.
Slide 47: ASPEN: Annualized Rate of Adjudicated Pulmonary Exacerbations
RESPIRATORY | Brensocatib (Bronchiectasis) ASPEN: Annualized Rate of Adjudicated Pulmonary Exacerbations Over 52 Weeks Primary endpoint 1.5 Annualized rate of exacerbationsa 21.1% Risk 19.4% Risk 1.286 reduction reduction 1 1.015 1.036 Brensocatib Brensocatib 10 mg 25 mg n=583 n=575 0.5 Rate ratio v
Slide 48: ARIKAYCE Phase 3 Programs to Potentially Expand MAC Indication
RESPIRATORY | ARIKAYCE ARIKAYCE Phase 3 Programs to Potentially Expand MAC Indication NCT04677543 & NCT04677569 Trial summaries Screening ARISE Primary Endpoint • Demonstrate reliability, validity and responsiveness of 99 Adults enrolled with new Months 1-6 Month 7 the patient reported outcome (PRO)
Slide 49: ARISE: QoL-B Respiratory Score Changes Show Clear Separation
RESPIRATORY | ARIKAYCE (Refractory MAC) ARISE: QoL-B Respiratory Score Changes* Show Clear Separation When PGI-S Category Improved vs. No Change or Decline Estimated Meaningful Score Difference Region is +12.2 to +16.7 points Estimated Meaningful Score Difference for QoL-B Resp. in MAC patients is +
Slide 50: ARISE: Comparison Between Treatment Arms Shows Favorable Trend for ARIKAYCE
RESPIRATORY | ARIKAYCE (Refractory MAC) ARISE: Comparison Between Treatment Arms Shows Clear Favorable Trend for ARIKAYCE Arm QoL-B Respiratory Domain ALIS+AZI+ETH ELC+AZI+ETH Difference N=48 N=51 # of Participants Evaluated for Change 43 48 from Baseline to Month 7 # of Missing Change from Baseline
Slide 51: ARISE: QoL-B Respiratory Score Changes from Baseline to Month 7
RESPIRATORY | ARIKAYCE (Refractory MAC) ARISE: QoL-B Respiratory Score Changes from Baseline to Month 7 Show Clear Separation for ARIKAYCE Group 100 90 Estimated Meaningful Score 80 Difference Median cfor hangeQoL-B Resp. among in patients MAC patients'somewhat responding is +14.8 points better' on
Slide 52: ARISE: Higher, More Persistent Culture Conversion in ARIKAYCE Arm
RESPIRATORY | ARIKAYCE (Refractory MAC) ARISE: Significantly* Higher, More Persistent Culture Conversion in ARIKAYCE Arm Percentage of Patients Achieving Culture Conversion, Months 6 and 7 90% Difference at Month 7 80% 80.6% Nominally Statistically 78.8% Significant* 70% (p-value = 0.0010) 60% 63.9%
Slide 53: ARISE: Patients in ARIKAYCE Arm Converted Faster
RESPIRATORY | ARIKAYCE (Refractory MAC) ARISE: For Those Who Achieved Culture Conversion, Patients in ARIKAYCE Arm Converted Faster Percentage of Patients with Two Consecutive Negative Cultures by Month 2, Of Those Who Converted By Month 6 80% ~74% of converters on ARIKAYCE 70% 74% achieved clinical
Slide 54: ARISE: Culture Conversion and QoL-B Respiratory Score Correlation
RESPIRATORY | ARIKAYCE (Refractory MAC) ARISE: Correlation Shown Between Culture Conversion and QoL-B Respiratory Score Changes in ARIKAYCE Arm ALIS+AZI+ETH (N=48) Culture Converted Not Culture Converted Difference by Month 6 by Month 6 Change in QoL-B Resp. Score +15.74 +3.53 +12.21 (Baseline to Mo
Slide 55: ARISE: No New or Unexpected Safety Signals
RESPIRATORY | ARIKAYCE (Refractory MAC) ARISE: No New or Unexpected Safety Signals in ARISE All serious TEAEs unrelated to ARIKAYCE ALIS+AZI+ETH ELC+AZI+ETH Total N=48 N=51 N=99 Study completion (%) 44 (91.7) 48 (94.1) 92 (92.9) Treatment completion with all 3 drugs (%) 35 (72.9) 47 (92.2) 82 (82.8)
Slide 56: Baseline Characteristics Were Well-Balanced Across Arms
RESPIRATORY | ARIKAYCE (MACLD) Baseline Characteristics Were Well-Balanced Across Arms ARIKAYCE Arm1 Comparator Arm2 Total N=213 N=212 N=425 Age: Median at Screening, Years 68.0 69.0 68.0 ≥65, % (n) 67.1% (143) 70.3% (149) 68.7% (292) Sex: Female, % (n) 80.3% (171) 78.3% (166) 79.3% (337) Geographic
Slide 57: RSS: Statistically Significant Improvement With ARIKAYCE at Month 13
RESPIRATORY | ARIKAYCE (MACLD) RSS: Statistically Significant Improvement in RSS1 Observed With ARIKAYCE at Month 13 PRIMARY ENDPOINT 3.11 points, p=0.0299* 20 95% CI [0.30, 5.92] 17.77 15 14.66 LS Mean Change 10 From Baseline RSS to Month 13† 5 SE (1.01) SE (1.01) 0 ARIKAYCE ALIS ArmArm2 Comparator
Slide 58: Responder Analysis: Higher Rates of Clinically Meaningful Improvement in RSS
RESPIRATORY | ARIKAYCE (MACLD) Responder Analysis: ARIKAYCE Arm Showed Higher Rates of Clinically Meaningful Improvement in RSS1 at Month 13 — ARIKAYCE Arm2 — Comparator Arm3 Estimated Range of Meaningful Within-Patient RSS Improvement† CDF (%) 8.2% Difference at higher threshold favoring 8.0% ARIKA
Slide 59: Culture Conversion by Month 13
RESPIRATORY | ARIKAYCE (MACLD) Culture Conversion: Statistically Significant Greater Proportion of ARIKAYCE Patients Achieved Culture Conversion1 by Month 13 SECONDARY ENDPOINT 100 26.8%-points, p<0.0001* 95% CI [18.3%, 35.3%] 80 82.4% 60 55.6% Rate of Culture Conversion (%) by Month 13† 40 20 SE (2
Slide 60: Durable Culture Conversion by Month 15
RESPIRATORY | ARIKAYCE (MACLD) Statistically Significant Greater Proportion of ARIKAYCE Patients Achieved Durable Culture Conversion1 by Month 15 KEY SECONDARY ENDPOINT 100 28.6%-points, p<0.0001* 80 95% CI [19.7%, 37.5%] 76.2% 60 Rate of Durable Culture Conversion (%) by Month 15† 40 47.6% 20 SE (3
Slide 61: Earlier and Greater Culture Conversion Rates at Every Timepoint
RESPIRATORY | ARIKAYCE (MACLD) Earlier and Greater Culture Conversion1 Rates Were Achieved in ARIKAYCE Arm at Every Measured Timepoint 100 ARIKAYCE Arm ARIKAYCE Arm 3 Comparator Arm Comparator Arm 4 80 Culture Conversion, ±SE (%)† Proportion of Patients with 60 40 20 71.5 43.6 80.7 53.1 84.0 56.4 84
Slide 62: Safety: No New or Unexpected Safety Signals Observed
RESPIRATORY | ARIKAYCE (MACLD) Safety: No New or Unexpected Safety Signals Observed ARIKAYCE Arm1 Comparator Arm2 N=213 N=212 DISPOSITION % n % n Study Completion** 90.6% 193 93.4% 198 ARIKAYCE/Comparator Treatment Completion 81.7% 174 88.2% 187 ARIKAYCE/Comparator Discontinuation 18.3% 39 11.8% 25
Slide 63: TPIP Phase 1 Study: Key Takeaways
RESPIRATORY | TPIP TPIP Phase 1 Study: Key Takeaways • Safety profile was generally well tolerated, AEs were mild and consistent with inhaled prostanoid • Tolerability was improved with an up-titration approach • Findings suggest TPIP may be safely dosed at nominal doses far in excess of TYVASO® • P
Slide 64: TPIP Phase 2a in PH-ILD
RESPIRATORY | TPIP (PH-ILD) TPIP Phase 2a in PH-ILD NCT05176951 Trial summary Treatment period PH-ILD Trial 4 weeks 4 weeks 16 weeks Primary Endpoints 3-week titration • Safety and tolerability period; last Steady-state dose increase at • Oxygenation at exercise period week 5 visit Secondary Endpoin
Slide 65: PH-ILD Phase 2 Study Met Primary Objective of Safety and Tolerability
RESPIRATORY | TPIP (PH-ILD) PH-ILD Phase 2 Study Met Primary Objective of Safety and Tolerability, With Lower Rates of Treatment Discontinuation and SAEs vs. Placebo Trial was randomized 3:1 TPIP vs. placebo TPIP (N=29) Placebo (N=10) Dose Titration % Patients Titrated to Maximum 640 µg Dose of TPIP
Slide 66: PH-ILD Phase 2 Exploratory Efficacy Endpoints Support Phase 3
RESPIRATORY | TPIP (PH-ILD) PH-ILD Phase 2 Exploratory Efficacy Endpoints Support Advancement Into Phase 3 Trial was randomized 3:1 TPIP vs. placebo TPIP (N=29) Placebo (N=10) Week 16 n Week 16 n 6-Minute Walk Distance (6MWD) 30 29 N/A 10 Treatment effect (TPIP vs. placebo) at Week 161 (m) [-49.0, 1
Slide 67: TPIP: Change From Baseline in Small and Large Blood Vessels
RESPIRATORY | TPIP (PH-ILD) TPIP: Change From Baseline in Small and Large Blood Vessels Lung imaging results from the Phase 2 PH-ILD study Baseline, mean (SD) Change from baseline, % Effect TPIP Placebo TPIP Placebo P value size,a % (n=9) (n=5) (n=9) (n=5) BV5A (mL) 19.5 (7.3) 33.6 (21.1) +14.0 –7.5
Slide 68: TPIP: Change From Baseline in High Attenuation Abnormality Score
RESPIRATORY | TPIP (PH-ILD) TPIP: Change From Baseline in High Attenuation Abnormality Scorea Lung imaging results from the Phase 2 PH-ILD study Change from baseline, mean (SD) Effect size,c % P value TPIP (n=9) Placebo (n=5) HAA score,b % –0.7 (2.7) 0.3 (1.8) –10.5 0.54 A numerical decrease from ba
Slide 69: TPIP: FRI in Representative Patients Treated With TPIP vs. Placebo
RESPIRATORY | TPIP (PH-ILD) TPIP: FRI in Representative Patients Treated With TPIP (A) vs. Placebo (B) Lung imaging results from the Phase 2 PH-ILD study TPIP (A) • TPIP treatment resulted in a significant increase in the fraction of blood volume in small arteries and a directional improvement in sm
Slide 70: TPIP Phase 2b in PAH
RESPIRATORY | TPIP (PAH) TPIP Phase 2b in PAH NCT05147805 Trial summary Treatment period 4 weeks 4 weeks PAH Trial (Week 16) 16 weeks 3-week titration Primary Endpoint period; last Steady-state • Change from baseline in pulmonary dose increase period vascular resistance (PVR)* at Week 5 visit 102 pa
Slide 71: Baseline Characteristics Reasonably Well-Balanced Across Study Arms
RESPIRATORY | TPIP (PAH) Baseline Characteristics Reasonably Well-Balanced Across Study Arms TPIP (N=69) Placebo (N=33) Total (N=102) Age: Mean, years (SD) 48.1 (15.00) 46.9 (15.22) 47.7 (15.00) Age < 65 years, % (n) 75.4 (52) 87.9 (29) 79.4 (81) Sex: Female, % (n) 84.1 (58) 78.8 (26) 82.4 (84) BMI:
Slide 72: TPIP Generally Well-Tolerated With a Low Discontinuation Rate
RESPIRATORY | TPIP (PAH) TPIP Generally Well-Tolerated With a Low Discontinuation Rate TPIP (N=69) Placebo (N=33) Total (N=102) TPIP Dose Titration, % (n)* Participants Titrated to at least 480 μg 84.1 (58) 84.8 (28) 75% Titrated to max dose of 640 μg 75.4 (52) 81.8 (27) Participants Completed the S
Slide 73: PVR: Highly Statistically Significant Primary Endpoint Achieved
RESPIRATORY | TPIP (PAH) PVR: Highly Statistically Significant Primary Endpoint Achieved With Once-Daily Therapy (P<0.001*) TPIP (N=69) Placebo (N=33) Week 16 n Week 16 n Primary Endpoint Pulmonary Vascular Resistance 35% PVR at Baseline: Mean PVR, Wood Units (SD) 9.588 (5.0072) 69 11.069 (5.9400) 3
Slide 74: Exercise Capacity: TPIP Showed a Clear Improvement in 6MWD
RESPIRATORY | TPIP (PAH) Exercise Capacity: TPIP Showed a Clear Improvement in 6MWD (P=0.003*) TPIP (N=69) Placebo (N=33) Week 16 n Week 16 n Secondary Endpoint All Efficacy Endpoints 6-Minute Walk Distance Measured ~24 Hours After Dose 35.5 6MWD at Baseline (m): Mean (SD) 348.48 (79.791) 69 371.06
Slide 75: Cardiac Stress: TPIP Showed a Meaningful Reduction in NT-proBNP
RESPIRATORY | TPIP (PAH) Cardiac Stress: TPIP Showed a Meaningful Reduction in NT-proBNP (P<0.001*) TPIP (N=69) Placebo (N=33) Week 16 n Week 16 n Secondary Endpoint NT-proBNP Concentration All Efficacy Endpoints Measured ~24 Hours After Dose Concentration at Baseline (pg/mL): 60% Mean 785.58 69 798
Slide 76: WHO Functional Class: More Patients on TPIP Improved
RESPIRATORY | TPIP (PAH) WHO Functional Class: More Patients on TPIP Achieved an Improvement in Functional Class TPIP (N=69) Placebo (N=33) Week 16 n Week 16 n Exploratory Endpoint WHO Functional Class Shift, % FC Improvement FC Improvement* at Week 16: 30.4 21 15.2 5 Week 16 vs. Baseline FC II Impr
Slide 77: Cardiac Index: TPIP Showed an Increase Compared to Placebo
RESPIRATORY | TPIP (PAH) Cardiac Index: TPIP Showed an Increase Compared to Placebo (P=0.006*) TPIP (N=69) Placebo (N=33) Week 16 n Week 16 n Exploratory Endpoint Cardiac Index, L/min/m2 TPIP Cardiac Index at Baseline: Cardiac Index 15% Mean 2.641 69 2.691 33 Cardiac Index at Week 16: Mean 3.070 62
Slide 78: Safety: Most Common TPIP TEAEs Consistent With Inhaled Treprostinil
RESPIRATORY | TPIP (PAH) Safety: Most Common TPIP TEAEs Were Consistent With Known Profile of Inhaled Treprostinil TPIP (N=69) Placebo (N=33) Week 16 n Week 16 n Safety TEAEs, % TPIP Any TEAE 88.4 61 75.8 25 Serious TEAE 7.2 5 3.0 1 Incidence Severe TEAE 5.8 4 3.0 1 TEAE Leading to Treatment Discont
Slide 79: Open-Label Extension Evaluating Long-Term Safety & Tolerability Over 24 Months
RESPIRATORY | TPIP (PAH) Open-Label Extension Intended to Evaluate TPIP’s Long-Term Safety & Tolerability Over 24 Months NCT05649748 Key OLE Study Endpoints Open-Label Treatment Period *** Primary Endpoint 16 weeks 24 months 4 weeks • Safety and tolerability (frequency & severity of TEAEs) 3-week ti
Slide 80: Baseline Characteristics Were Comparable Across Groups
RESPIRATORY | TPIP (PAH) Baseline† Characteristics Were Comparable Across Groups Continued TPIP1 Placebo Crossed2 Total N=60 N=31 N=91 Age: Mean, years 47.9 47.6 47.8 Sex: Male, % (n) 13.3% (8) 19.4% (6) 15.4% (14) Geographic Region: % (n) United States 10.0% (6) 9.7% (3) 9.9% (9) Europe 36.7% (22)
Slide 81: TPIP Generally Safe and Well-Tolerated Through Month 12
RESPIRATORY | TPIP (PAH) TPIP Was Generally Safe and Well-Tolerated; No Newly Identified Safety Signals Through Month 12 Total (N=91) SAFETY % n Any TEAE 89.0% 81 ≤640 µg Max Dose Achieved 86.1% 62 All TEAEs of cough were >640 µg Max Dose Achieved** 89.5% 17** classified as mild or moderate, Serious
Slide 82: Sustained Improvement in 6MWD Over 12 Months
RESPIRATORY | TPIP (PAH) Patients that Continued on TPIP Showed Sustained Improvement in 6MWD Over 12 Months Continued T PIP Mean-Continued T PIP Median-Continued T PIP Placebo-crossed Continued TPIP Placebo Crossed Mean-Placebo-crossed Median-Placebo-crossed Mean Improvement from Mean - Continued T
Slide 83: Sustained Reduction in NT-proBNP Over 12 Months
RESPIRATORY | TPIP (PAH) Patients that Continued on TPIP Showed Sustained Reduction in NT-proBNP Over 12 Months 1. 5 Phase 2b Study Mean Reduction from Basel i ne * Continued TPIP 202 Baseline NT-proBNP Concentration† (SE) Placebo Crossed Baseline* NT-proBNP Concentration at Month 12 wi t h Geomet r
Slide 84: Meaningful Improvement in WHO Functional Class by Month 12
RESPIRATORY | TPIP (PAH) Both Groups Showed Meaningful Improvement in WHO Functional Class (FC) by Month 12 Continued TPIP (N=60) Placebo Crossed (N=31) Phase 2b Study Phase 2b Study Functional Class 100% by Month 12 Proportion of Patients† 75% >25% of all patients 50% achieved FC I − FC I indicates
Slide 85: REVEAL Lite 2.0 Score: Simplified Validated Tool to Estimate Mortality Risk
RESPIRATORY | TPIP (PAH) REVEAL Lite 2.0 Score: Simplified, Non-Invasive, Validated Tool to Estimate the Mortality Risk of PAH Patients Estimated Estimated Scoring1,2 Points Mortality Risk Morbidity Risk3 A 1-point improvement from High Risk ≥8 >10% at 1 year baseline REVEAL score is associated with
Slide 86: Clinically Meaningful Improvement in Mean REVEAL Lite 2.0 Score by Month 12
RESPIRATORY | TPIP (PAH) Both Groups Showed Clinically Meaningful Improvement in Mean REVEAL Lite 2.0 Score by Month 12 Continued TPIP Placebo Crossed 6. 0 Phase 2b Study Intermediate Risk REVEAL Score (SE)† Status (>5 to <8) Improvement Mean REVEAL Li t e 2. 0 Sc or e ( LOCF) 5. 5 d Er Score by Mon
Slide 87: TPIP Has the Potential for Clear Differentiation in PH-ILD and PAH
RESPIRATORY | TPIP (PAH) TPIP Has the Potential for Clear Differentiation in PH-ILD and PAH Approved Treprostinil Therapies*** TYVASO® Orenitram® Potential TPIP Remodulin® & Yutrepia® & Uptravi® Differentiators Convenience* Method of Inhaled Inhaled IV or administration (nebulized and/or Oral (dry p
Slide 88: Epidemiological Footnotes (1 of 3)
Epidemiological Footnotes (1 of 3) 1. Internal analysis of published BE epidemiology, including internal market research and US patient level claims data analysis: a) Weycker, et al. Prevalence and incidence of NCFBE among US adults in 2013. Chronic Respiratory Disease. 2017 b) BE Patient Level Clai
Slide 89: Epidemiological Footnotes (2 of 3)
Epidemiological Footnotes (2 of 3) 4. Internal analysis of published NTM epidemiology, including internal market research and US patient level claims data analysis: a) Jennifer Adjemian, Kenneth N Olivier, Amy E Seitz, Steven M Holland, D Rebecca Prevots: Prevalence of nontuberculous mycobacterial l
Slide 90: Epidemiological Footnotes (3 of 3)
Epidemiological Footnotes (3 of 3) 6. Internal assessment of published epidemiology, including: a) Andersen, C. U., Mellemkjær, S., Hilberg, O., Nielsen-Kudsk, J. E., Simonsen, U., & Bendstrup, E. (2012). Pulmonary hypertension in interstitial lung disease: prevalence, prognosis and 6 min walk test.
Slide 91: TPIP Footnotes (1 of 3)
TPIP Footnotes (1 of 3): 1. Insmed Phase 2b Study of TPIP in PAH; Placebo-Adjusted Reduction from Baseline PVR at Week 16; Analysis performed using an ANCOVA model, adjusting for treatment group, baseline pulmonary vascular resistance (PVR), and randomization stratification factors. The model was ap
Slide 92: TPIP Footnotes (2 of 3)
TPIP Footnotes (2 of 3): 9. Phase 3 TRIUMPH study; Hodges-Lehmann (H-L) median treatment difference in change from baseline 6MWD at 12 weeks measured at peak drug exposure (p<0.001); McLaughlin, V, Benza, R, Rubin, L. et al. Addition of Inhaled Treprostinil to Oral Therapy for Pulmonary Arterial Hyp
Slide 93: TPIP Footnotes (3 of 3)
TPIP Footnotes (3 of 3): 16. SOTERIA OLE study; Supplementary Table 5. Change from Baseline at Week 24 and Year 1; Mean change from baseline defined as measurement at Visit 1 of SOTERIA or the last measure from the parent study before rollover in 6MWD; Interim results of SOTERIA; Preston, I. R., Bad
Slide 94: Launch Analog Footnotes (1 of 3)
Launch Analog Footnotes (1 of 3) 1. BRINSUPRI | Launched in Q3 2025; Q4 2025 represents the first full quarter of sales a) BRINSUPRI generated US$144.6 million in net product sales during the fourth quarter of 2025 (Insmed Incorporated, 2026). Insmed Incorporated. Insmed Reports Fourth-Quarter and F
Slide 95: Launch Analog Footnotes (2 of 3)
Launch Analog Footnotes (2 of 3) 4. DUPIXENT | Launched in Q2 2017 a) Dupixent collaboration revenue was US$28.6 million during the second quarter of 2017 (Regeneron Pharmaceuticals, Inc., 2017). Regeneron Pharmaceuticals, Inc. (2017, August 3). Quarterly report (Form 10-Q) for the quarterly period
Slide 96: Launch Analog Footnotes (3 of 3)
Launch Analog Footnotes (3 of 3) 7. TEZSPIRE | Launched in Q1 2022 a) TEZSPIRE generated US$7 million in sales during the first quarter of 2022 (Amgen Inc., 2022). Amgen reports first quarter 2022 financial results. https://investors.amgen.com/news- releases/news-release-details/amgen-reports-first-
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