Dianthus Therapeutics, Inc. · NASDAQ:DNTH
Dianthus Therapeutics — Investor Presentation (August 2026)
97 slides from Dianthus Therapeutics's investor presentation, August 2026
Slide 1: Advancing a leading autoimmune-focused company
Advancing a leading autoimmune-focused company August 2026
Slide 2: Forward-looking statements
Forward-looking statements Certain statements in this presentation, other than purely historical information, may constitute “forward-looking statements” within the meaning of the federal securities laws, including for purposes of the safe harbor provisions under the United States Private Securities
Slide 3: Advancing a leading autoimmune-focused company with neuromuscular and rheumatology franchises
Advancing a leading autoimmune-focused company with neuromuscular and rheumatology franchises Advancing a portfolio of autoimmune therapeutics with best-in-disease, pipeline-in-a-product potential and targeting patient-friendly, infrequent S.C. self-administration Claseprubart: Neuromuscular Franchi
Slide 4: Two clinical-stage candidates with best-in-disease, pipeline-in-a-product potential
Two clinical-stage candidates with best-in-disease, pipeline- in-a-product potential Claseprubart DNTH212 gMG CIDP MMN SjD, SLE and DM Indications >100,000 U.S. patients >40,000 U.S. patients >10,000 U.S. patients ~625,000 U.S. patients Multi-billion $, growing market Targets both innate and adaptiv
Slide 5: Claseprubart: Building a Best-in-Disease Neuromuscular Franchise
Claseprubart: Building a Best-in-Disease Neuromuscular Franchise
Slide 6: Building on the promise of claseprubart to be a potential pipeline-in-a-product
Building on the promise of claseprubart to be a potential pipeline-in-a-product and best-in-disease therapy in growing and underserved markets Ph.2 MaGic Showed Rapid & Q3’25 Robust Improvements in gMG CAPTIVATE Part A Early Q1’26 GO Decision in CIDP Ph. 2 MoMeNtum Top-line Data Anticipated Dec. ’26
Slide 7: Claseprubart has opportunity to compete as a potential 1L biologic in three large markets
Claseprubart has opportunity to compete as a potential 1L biologic in three large and growing US neuromuscular markets >150K patients >10K MMN patients >140K patients POC supporting Classical Pathway inhibition and limited competition >40K CIDP patients Ph. 2 top-line data Potential for aC1s inhibit
Slide 8: Claseprubart: Opportunity in Generalized Myasthenia Gravis
Claseprubart: Opportunity to be a Best-in- Disease, First-Line Biologic for Generalized Myasthenia Gravis
Slide 9: Ultomiris is the leading blockbuster complement inhibitor
Ultomiris is the leading blockbuster complement inhibitor, with continued growth driven by first-line biologic use in gMG Ultomiris sales grew 12% in Q2’26… “driven by patient C5 Inhibitors (Ultomiris & Soliris) demand across indications, including the competitive MG and Global Sales ($M) PNH market
Slide 10: The US gMG market has significant potential to expand
The US gMG market has significant potential to expand as <20% of AChR+ patients1 are treated with biologics Significant opportunity for a highly differentiated, more patient-friendly biologic to expand use of biologics in gMG Current US gMG Market >$8B gMG Opportunity for Complement in 20352 C5s Est
Slide 11: Survey of US Neurologists confirms opportunity in gMG
Survey of US Neurologists confirms significant opportunity for differentiated biologic in gMG market Claseprubart aims to address the significant unmet needs in the gMG market ~81% ~78% Total Neurologists 81 Neuromuscular specialists 67% Generalists 33% of Neurologists of Neurologists believe patien
Slide 12: MaGic Ph. 2 Results Support a Potentially Best-in-Disease Treatment for gMG
MaGic Ph. 2 Results Support a Potentially Best-in-Disease Treatment for gMG
Slide 13: MaGic Ph. 2 top-line results support a potentially best-in-disease treatment for gMG
MaGic Ph. 2 top-line results support a potentially best-in- disease treatment for gMG Efficacy Endpoints Safety Endpoints Optimal Dose Strong results support claseprubart Generally well tolerated, with a Comparable efficacy & safety across potential as a best-in-disease potentially differentiated sa
Slide 14: MaGic is a global Ph. 2 trial in AChR+ gMG patients
MaGic is a global Ph. 2 trial in AChR+ gMG patients A global, multicenter, randomized, double-blind, placebo-controlled study to evaluate the safety, efficacy, and PK / PD of claseprubart administered S.C. following initial loading dose Highlights • Design: ~60 male and female subjects 13-week RCT r
Slide 15: Claseprubart arms demonstrated rapid, sustained improvements in MG-ADL score
Claseprubart arms demonstrated rapid, sustained, and clinically meaningful improvements in MG-ADL score 0.0 Placebo (n=22) Claseprubart 300mg Q2W (n=21) Claseprubart 600mg Q2W (n=22) -1.0 Mean Change from Baseline in MG-ADL Score -2.0 -2.8 -3.0 -1.8 -2.6 P=0.0113* P=0.0006* -4.0 -4.6 -5.0 -5.4 -6.0
Slide 16: Claseprubart arms demonstrated rapid, sustained improvements in QMG score
Claseprubart arms demonstrated rapid, sustained, and clinically meaningful improvements in QMG score 0.0 Placebo (n=22) Claseprubart 300mg Q2W (n=21) Claseprubart 600mg Q2W (n=22) Mean Change from Baseline -1.0 -2.0 in QMG Score -2.0 -2.4 -2.5 P=0.0144* P=0.0111* -3.0 -4.0 -4.4 -4.5 -5.0 Statistical
Slide 17: >60% achieved ≥5 point improvement in MG-ADL
>60% of participants on claseprubart 300mg/2mL achieved ≥5 point improvement in MG-ADL Improvement in MG-ADL Total Score Claseprubart 300mg/2mL Q2W Placebo Claseprubart 600mg/4mL Q2W Placebo ≥11 ≥11 5% ≥10 ≥10 5% Improvement from Baseline ≥9 11% ≥9 10% in MG-ADL Score ≥8 11% ≥8 14% ≥7 28% ≥7 29% ≥6
Slide 18: >60% achieved ≥5 point improvement in QMG
>60% of participants on claseprubart 300mg/2mL achieved ≥5 point improvement in QMG Improvement in QMG Total Score Claseprubart 300mg/2mL Placebo Claseprubart 600mg/4mL Placebo ≥13 6% ≥13 ≥12 6% ≥12 Improvement from Baseline ≥11 6% ≥11 ≥10 17% 5% ≥10 5% in QMG Score ≥9 22% 5% ≥9 10% 5% ≥8 22% 14% ≥8
Slide 19: 37% achieved Minimal Symptom Expression on MG-ADL at Week 13
37% of 300mg/2mL claseprubart-treated participants achieved Minimal Symptom Expression on MG-ADL at Week 13 Minimal Symptom Expression (MSE) % of Participants Achieving MG-ADL Score of 0 or 1 at Week 13 P=0.1031 P=0.0550* 37% 27% 14% Placebo Claseprubart 300mg Claseprubart 600mg (Week 13) (Week 13)
Slide 20: Across key efficacy measures, claseprubart demonstrated robust responses
Across key efficacy measures, claseprubart demonstrated robust and clinically meaningful responses Claseprubart Claseprubart 300mg/2mL Q2W 600mg/4mL Q2W Placebo Placebo- Placebo- Absolute Absolute adjusted adjusted MG-ADL mean change from -1.8 -2.6 -2.8 -4.6 -5.4 baseline at Week 13 (P=0.0113)* (P=0
Slide 21: Claseprubart was generally well tolerated in Ph. 2
Claseprubart was generally well tolerated, with a favorable, potentially differentiated safety profile in Ph. 2 Placebo Claseprubart 300mg/2mL Q2W Claseprubart 600mg/4mL Q2W (N=22) (n=21) (n=22) Clinical adverse events (AEs)1 11 (50.0%) 13 (61.9%) 15 (68.2%) Related serious AEs 1 (4.5%) 0 (0%) 0 (0%
Slide 22: Rationale for Q4W 300mg/2mL Dosing in gMG
Rationale for Q4W 300mg/2mL Dosing & Potential for Enhanced, Best-in-Disease Efficacy in gMG
Slide 23: OLE data support addition of 300mg/2mL Q4W in Ph. 3
OLE data support addition of 300mg/2mL Q4W in Ph. 3 PK levels approximately half of 300mg/2mL Q2W steady state resulted in robust reductions on MG-ADL & QMG Mean Change in PBO Patients’ MG-ADL and QMG Score from RCT Baseline to OLE Week 6 Estimated PK QMG MG-ADL QMG: 14.2 of ~65 µg/mL 14.0 PBO Patie
Slide 24: Adding QMG screening criteria in Ph. 3 may better control placebo response
Adding QMG screening criteria in Ph. 3, similar to zilucoplan Ph. 31, may better control for placebo response Claseprubart MG-ADL Change from Baseline Zilucoplan Ph. 3 MG-ADL Change from Baseline in Patients All Patients Patients with QMG ≥10 with QMG ≥12 Placebo Claseprubart 300mg Q2W Placebo Clase
Slide 25: Potential for improved efficacy vs. C5 inhibitors due to upstream inhibition
Potential for improved efficacy vs. C5 inhibitors with claseprubart may be due to upstream inhibition Claseprubart Prevents the Creation of Pro-inflammatory Split Products C3a and C3b vs. Ravulizumab C3a Formation1 C3b Deposition2* 10 100 % of hRBC with C3b Deposition 0 80 % Reduction in C3a -20 60
Slide 26: EMERGE is a global Ph. 3 trial evaluating Q4W and Q2W claseprubart
EMERGE is a global Ph. 3 trial evaluating Q4W and Q2W S.C. claseprubart in AChR+ gMG; top-line data 2H’28 No ANA screening exclusion criteria or routine ANA testing during RCT or OLE, inclusion criteria of MG-ADL of ≥6 and QMG of ≥10, and 17-week S.C. treatment period per alignment with FDA Highligh
Slide 27: Achieving this profile could position claseprubart as best-in-disease for gMG
Achieving this profile could position claseprubart as a potential best-in-disease treatment for gMG C5 OR SUPERIOR EFFICACY C1s SAFETY AUTOINJECTOR CONVENIENCE (ULTOMIRIS/SOLIRIS/ZILBRYSQ) (ENJAYMO) (DUPIXENT) Similar or superior MG-ADL to FDA- Comparable safety to FDA-approved Comparable convenienc
Slide 28: Claseprubart: Opportunity in Chronic Inflammatory Demyelinating Polyneuropathy
Claseprubart: Opportunity to Change the Treatment Paradigm in Chronic Inflammatory Demyelinating Polyneuropathy
Slide 29: The US CIDP market offers substantial growth potential
The US CIDP market offers substantial growth potential given high unmet need and limitations of current standard of care Opportunity for an active C1s inhibitor with the target profile of claseprubart to replace the standard of care Current US CIDP Market >$8B CIDP Opportunity for Complement in 2035
Slide 30: Survey of US Neurologists supports opportunity in CIDP
Survey of US Neurologists supports potential transformative opportunity in CIDP Claseprubart aims to differentiate and effectively address the significant unmet needs in the CIDP market Total Neurologists 80 Neuromuscular Specialist 81% ~79% ~66% Generalist 19% of Neurologists strongly believe of Ne
Slide 31: Surveyed Neurologists want better options than IVIg for CIDP
Surveyed Neurologists want safer, more effective and convenient treatment options than IVIg for CIDP patients Overall, surveyed Neurologists believe ~50% patients on IVIg have partial or no response to treatment % of Surveyed Neurologists Who Strongly Believe… 75% Significant unmet need for effectiv
Slide 32: Ph. 3 CAPTIVATE trial Part B top-line guidance expected by YE'26
Ph. 3 CAPTIVATE trial Part B top-line guidance expected by YE’26 PART B: 52-Week Pbo- Highlights controlled RCT Responders Randomized 1:1 • Design: All subjects receive claseprubart in PART A: Up to 13-Week Extension: 104 Weeks Part A for up to 13 weeks. Only responders Open-Label Claseprubart Only
Slide 33: Significant differences between CAPTIVATE and ADHERE
Significant differences between CAPTIVATE and ADHERE Efgartigimod (FcRn) Claseprubart (aC1s) Considerations Key Differentiators of CAPTIVATE S.C. QW 300mg/2mL S.C. Q2W Evaluating claseprubart in SoC- Refractory CIDP patients, in Ph. 3 Study SoC-Treated SoC-Treated addition to a broader CIDP patient
Slide 34: Active C1s inhibition with riliprubart has demonstrated clinical proof-of-concept
Active C1s inhibition with riliprubart has demonstrated clinical proof-of-concept across broad patient groups Ph. 2 Riliprubart Data in Active C1s in CIDP1 with High Volume, Weekly Dosing of 600mg/4mL2 Improvement Data not seen without Ig with FcRns washout Claseprubart is being evaluated with a con
Slide 35: Claseprubart in vitro affinity and potency
Claseprubart in vitro affinity and potency Affinity assays Functional assays of Classical Pathway inhibition Fold DNTH103 riliprubart Improvement Binding CH50 assay of RBC lysis Affinity KinExa 9pM 75pM ~8X in human serum3 Wieslab Classical Pathway Liposome lysis to human active Assay in human serum
Slide 36: 75% response rate in Interim Responder Analysis of CAPTIVATE Part A
75% response rate observed in Interim Responder Analysis from first 40 participants completing Part A of CAPTIVATE Breakdown of First 40 Participants Summary of Change from Baseline at Last Evaluable Visit1 Completing CAPTIVATE Part A Confirmed Responders Non-Responders 12.5% (N=30) (N=10) Stable 12
Slide 37: CAPTIVATE Part A baseline characteristics similar to precedent aC1s Ph. 2
CAPTIVATE Part A baseline patient group characteristics are similar to precedent aC1s Ph. 2 study1 Riliprubart Ph. 21 CAPTIVATE Part A2 Baseline Patient Group Characteristics (N=78) Baseline Patient Group Characteristics (N=40) ~10% ~15% SoC- SoC- Naïve Naïve ~23% ~23% SoC-Refractory SoC-Refractory
Slide 38: Target profile could position claseprubart as a blockbuster treatment for CIDP
Achieving this target profile could position claseprubart as a potential blockbuster treatment for CIDP EFFICACY C1s SAFETY AUTOINJECTOR CONVENIENCE (IVIg/SCIg) (ENJAYMO) (DUPIXENT) Improvement over SoC (i.e. Ig) with Comparable safety to FDA-approved Most convenient therapy with continuous, effecti
Slide 39: Claseprubart: Opportunity in Multifocal Motor Neuropathy
Claseprubart: Opportunity to be Best-in-Disease in Multifocal Motor Neuropathy
Slide 40: The US MMN market is a growing blockbuster opportunity
The US MMN market is a growing blockbuster market opportunity for claseprubart Opportunity for the target profile of claseprubart to become the new standard of care in MMN Current US MMN Market ~$4B MMN Opportunity for Complement in 20353 Standard Claseprubart Estimated US complement and Ig penetrat
Slide 41: Survey of US Neurologists supports MMN underdiagnosis and unmet need
Survey of US Neurologists supports MMN underdiagnosis and large unmet need Claseprubart aims to offer a uniquely effective, safe, convenient Classical Pathway inhibitor Total Neurologists 80 Neuromuscular Specialist Generalist 81% 19% ~81% ~73% of Neurologists strongly of Neurologists strongly belie
Slide 42: Over half of surveyed neurologists agree IVIg is suboptimal for MMN
Over half of surveyed US Neurologists agree IVIg is a suboptimal treatment option for MMN patients The lack of satisfaction with IVIg efficacy in MMN presents a significant opportunity for claseprubart % of Surveyed Neurologists Who Strongly Believe… 58% Patients on IVIg often report residual or una
Slide 43: Classical Pathway inhibition has demonstrated PoC in MMN
Classical Pathway inhibition has demonstrated PoC in MMN Empasiprubart (an inhibitor of both the Classical and Lectin Pathways) Ph. 2 Data Demonstrating Efficacy Signals1 “We hypothesize that targeting the classical complement pathway is a potential therapeutic approach in MMN. We investigated the i
Slide 44: Claseprubart demonstrates superior Classical Pathway potency vs. empasiprubart
Claseprubart demonstrates superior Classical Pathway potency vs. empasiprubart Empasiprubart Published Classical Pathway Potency Data Using Claseprubart Demonstrates Superior Classical Pathway the Quidel MicroVue CH501 Potency Head-to-head vs. Empasiprubart Using Same Assay ~38X more potent at IC90
Slide 45: Ph. 2 MoMeNtum top-line data in MMN anticipated December '26
Ph. 2 MoMeNtum top-line data in MMN anticipated in December ’26 A global, multicenter, randomized, double-blind, placebo-controlled study to evaluate the safety, efficacy, and PK / PD of claseprubart administered S.C. following initial loading dose Highlights • Design: 36 participants randomized to
Slide 46: Key differences between ARDA and MoMeNtum trials
Key differences between ARDA and MoMeNtum trials make cross-trial comparisons challenging Empasiprubart (C2) Claseprubart (aC1s) Considerations Key Attributes of MoMeNtum ARDA Trial MoMeNtum Trial Small study designed to demonstrate PoC and Total Participants = 54 Total Participants = 46 support ini
Slide 47: Claseprubart can address the unmet need in MMN with its target product profile
Claseprubart has the potential to address the unmet need in the MMN market with its unique target product profile Considerations Empasiprubart (C2)* Claseprubart (active C1s)* Key Differentiators of Claseprubart Claseprubart has ~6x more potent than empasiprubart MMN is an IgM and Published classica
Slide 48: Target profile could position claseprubart as a blockbuster treatment for MMN
Achieving this target profile could position claseprubart as a potential blockbuster treatment for MMN EFFICACY C1s SAFETY AUTOINJECTOR CONVENIENCE (IVIg/SCIg) (ENJAYMO) (DUPIXENT) Improvement over SoC (i.e., Ig) with Comparable safety to FDA-approved Most convenient therapy with continuous, effecti
Slide 49: DNTH212: Potential Best-in-Disease Bispecific Fusion Protein
DNTH212: Potential Best-in-Disease Bispecific Fusion Protein for Multiple Autoimmune Indications
Slide 50: DNTH212 is a bifunctional BDCA2 and BAFF/APRIL inhibitor
DNTH212 is a bifunctional BDCA2 and BAFF/APRIL inhibitor targeting two validated pathways Anti-BDCA2 Antibody Potent inhibition of Type 1 interferon • Inhibiting BDCA2 reduces Type 1 interferon TACI-CRD2 Domain production from plasmacytoid dendritic Potent reduction of Ig levels via cells (pDCs) BAF
Slide 51: Targeting both the innate and adaptive immune systems
Potential to drive superior clinical efficacy by targeting both the innate and adaptive immune systems Innate Immune System: Plasmacytoid Dendritic Cells (pDCs) Adaptive Immune System: B Cells • Key cell type producing Type 1 interferon • Generate autoantibodies, forming immune complexes that trigge
Slide 52: DNTH212 achieves superior pDC depletion compared to litifilimab in vitro
DNTH212 achieves superior pDC depletion compared to litifilimab in vitro Comparable Suppression of Pro-Inflammatory Deeper Depletion of pDC2 Cytokine IFN-α1 1200 400 DNTH212 DNTH212 900 300 Litifilimab Litifilimab pDC Counts IFN-α（pg/ml） 600 200 300 100 0 0 0.000001 0.0001 0.01 1 100 10000 0.000001
Slide 53: DNTH212 shows superior Ig inhibition compared to povetacicept in NHPs
DNTH212 shows superior inhibition of IgM, IgA, and IgG compared to povetacicept following single dose in NHPs IgM IgA IgG S.C. DNTH212 25 25 Mean% change from baseline 25 DNTH212 (1mg/kg S.C.) Mean% change from baseline 1 mg/kg Mean% change from baseline 0 0 0 DNTH212 10 mg/kg (10mg/kg S.C.) 24% -25
Slide 54: Priority indications with high unmet need and dual involvement
Priority indications with high unmet need and dual involvement of both innate and adaptive immune systems Prioritization Considerations DNTH212 Priority Clinical evidence or biological rationale of Rheumatology Indications both interferon / pDC and B-cell involvement Sjögren’s disease (SjD) ~350,000
Slide 55: SLE: Strong BDCA2 and BAFF/APRIL validation
SLE: Strong BDCA2 and BAFF / APRIL validation with a proven development path support DNTH212 opportunity Disease SLE is a systemic autoimmune disease affecting multiple organs such as the kidneys, skin, and joints Overview and may lead to significant or life-threatening end-organ damage BDCA2/Type 1
Slide 56: SLE: Potential for dual mechanism to address key unmet needs
SLE: Potential for dual mechanism to address key unmet needs despite approved treatments Litifilimab Ph. 2 data1 and multiple approvals (Saphnelo, Significant Unmet Need Benlysta) support dual-mechanism approach in SLE • SLE is a heterogenous disease - potential for dual-mechanism approach to impact
Slide 57: SjD: High unmet need for a single therapy
SjD: High unmet need for a single therapy that can address multiple disease manifestations Disease Sjögren’s destroys mucosal exocrine glands causing extreme dry eyes and mouth (sicca) and leads Overview to debilitating systemic symptoms such as fatigue and joint pain BDCA2/Type 1 IFN (Innate) BAFF/
Slide 58: SjD: Potential for increased efficacy from dual-mechanism approach
SjD: Potential for increased efficacy from dual-mechanism approach vs. targeting BAFF receptor only Ianalumab Ph. 31 highlights potential for B-cell Significant Unmet Need approaches to lower ESSDAI • Fatigue, pain and cognitive dysfunction are significant drivers of patient disability2 • 70% increa
Slide 59: DM: High unmet need for a single therapy
DM: High unmet need for a single therapy that can more effectively address the disease Disease DM causes progressive muscle weakness, painful rash, pruritus and interstitial lung disease; Overview limited therapeutic options beyond corticosteroids (i.e. IVIg) BDCA2/Type 1 IFN (Innate) BAFF/APRIL (Ad
Slide 60: DM: Dual-mechanism approach may more effectively address the disease
DM: Dual-mechanism approach may more effectively address the disease Dazukibart Ph. 2 data1 and IVIg2 lays foundation for Significant Unmet Need multi-organ efficacy in DM Dazukibart Ph. 2 IVIg IIT • Muscle weakness, fatigue and pain remain leading causes of morbidity3 • Limited therapeutic treatmen
Slide 61: Ph. 1 study initiated in China in Dec. '25, healthy volunteer data by YE'26
Ph. 1 study initiated in China in Dec. ’25, with healthy volunteer data by YE’26 Healthy Volunteers (Part A) ~46 HVs enrolled into seven cohorts: Healthy Volunteers (Part A) SLE Patients (Part B) • Treated (N= up to 6) S.C. Administration S.C. Administration • Placebo (N= up to 2) 1080mg N = 6:2 Dos
Slide 62: DNTH212 TPP aims to deliver superior efficacy, safety and convenience
DNTH212 TPP aims to deliver superior efficacy in a safe and well-tolerated therapy with patient friendly convenience EFFICACY SAFETY CONVENIENCE Bifunctional approach has Inhibiting Type 1 interferon or Targeting patient friendly S.C. potential for superior efficacy in BAFF/APRIL has been self-admin
Slide 63: Achieving DNTH212 TPP could position it as first-line, best-in-disease therapy
Achieving DNTH212 TPP could position DNTH212 as a first- line, best-in-disease therapy across multiple indications Clinically Superior Patient-friendly Favorable Pipeline-in-a- Robust IP Validated MoA Efficacy Convenience Safety Potential Product Protection Type 1 interferon and Targeting innate and
Slide 64: DNTH312: Expanding our leadership position across severe autoimmune diseases
DNTH312: Expanding our leadership position across severe autoimmune diseases
Slide 65: DNTH312 is a first-in-class next-generation bifunctional fusion protein
DNTH312 is a first-in-class, next-generation bifunctional fusion protein combining claseprubart and TACI targeting two complementary, validated pathways IgG1 Fc Claseprubart Potent Classical Pathway inhibition No effector function (“silent”) via aC1s TACI-CRD2 Domain YTE Mutation on Fc High affinity
Slide 66: Goal to drive superior efficacy by targeting both aC1s and BAFF/APRIL
Goal to drive superior clinical efficacy by targeting both aC1s and BAFF/APRIL Upstream Classical Pathway or aC1s inhibition results in: • Reduction of immune-complex-mediated tissue damage driven by MAC formation • Reduction in recruitment and activation of inflammatory cells (including B cells) th
Slide 67: DNTH312 builds on claseprubart and expands leadership in neuromuscular markets
DNTH312 builds on claseprubart best-in-class profile and expands Dianthus’ leadership in neuromuscular markets Initial Focus: Future Opportunity: Grow Leadership Position in Neuromuscular Diseases Expand into Other Therapeutic Areas Seeks to provide more benefit beyond claseprubart Leverages dua
Slide 68: DNTH312 demonstrates comparable in vitro potency and aC1s inhibition to claseprubart
DNTH312 demonstrates comparable in vitro potency and aC1s inhibition to potentially best-in-class claseprubart Functional Assays of Classical Pathway Inhibition Direct RBC Hemolysis CH50 Assay of RBC Lysis Wieslab Classical Pathway in Human Serum in Human Serum Assay in Human Serum 100 200 100 % Inh
Slide 69: DNTH312 shows similar Ig reduction vs. povetacicept in NHPs
DNTH312 shows similar depth of IgM, IgA, and IgG reduction vs. povetacicept following single dose in NHPs IgM IgM IgA IgGIgG 20 20 20 % Change from Baseline % Change from Baseline % Change from Baseline 10 10 10 DNTH312 0 0 0 -10 -10 -10 Maximal Ig Inhibition -20 -20 -20 -30 -30 ~58% -30 DNTH312 Pov
Slide 70: DNTH312 aims to be Phase 1 ready by YE'27
DNTH312 aims to be Phase 1 ready by YE’27 Key Benefits of DNTH312 Clinically validated MoAs Comparable NHP Comparable in vitro aC1s Comparable Ig reductions combined have the half-life to inhibition and potency to to povetacicept with a potential for superior claseprubart claseprubart longer half-li
Slide 71: Recap of Dianthus Leadership in Severe Autoimmune Diseases
Recap of Dianthus Leadership in Severe Autoimmune Diseases
Slide 72: Building the leading autoimmune-focused biotech through best-in-disease innovation
Building the leading autoimmune-focused biotech through potential best-in-disease innovation DNTH312: Next-generation Leadership Leverage Core Competencies to Expand Leadership Across Severe Autoimmune DNTH212: Expansion into Rheumatology SjD SLE DM U.S. Prevalence: ~625K Claseprubart: Foundation in
Slide 73: Advancing a leading autoimmune-focused biotech with two clinical stage programs
Advancing a leading autoimmune-focused biotech with two clinical stage programs Program Indication Preclinical Ph. 1 Ph. 2 Ph. 3 Upcoming Milestones gMG* • Ph. 3 top-line data expected in 2H’28 >100,000 U.S. Patients • Part B top-line guidance expected by YE’26 Claseprubart CIDP • Peer Milestone: ri
Slide 74: Appendix
Appendix
Slide 75: C1s is a clinically validated target in the Classical Complement Pathway
C1s is a clinically validated target in the Classical Complement Pathway with an FDA approved therapy 1 C1q Classical Pathway C1s is the only target of the The only pathway activated by the C1 complex with an FDA presence of IgG and IgM, which bind 2 approved therapy 3 to the C1 complex Enjaymo, FDA
Slide 76: Selectively targeting classical pathway aims to provide a safer complement inhibitor
Selectively targeting classical pathway aims to provide effective but safer complement inhibitor Targeting aC1s aims to deliver efficacy demonstrated with terminal inhibitors while preserving the critical immune activity of lectin and alternative pathways, leading to a lower risk of infection and no
Slide 77: Participant baseline characteristics were generally well balanced across arms
Participant baseline characteristics were generally well balanced across arms Claseprubart Claseprubart Placebo 300mg/2mL Q2W 600mg/4mL Q2W (N=22) AChR+ gMG participants (N=21) (N=22) Age, mean (SD), years 52.2 (16.5) 57.1 (13.7) 55.3 (12.0) Male, n (%) 13 (59%) 14 (67%) 10 (45%) Weight, mean (SD),
Slide 78: Statistically significant improvement in MG-ADL score at Week 13
Statistically significant improvement in MG-ADL score for both claseprubart arms vs. placebo at Week 13 Mean Change in MG-ADL Score from Baseline at Week 13 Placebo Claseprubart 300mg Q2W Claseprubart 600mg Q2W (n=22) (n=21) (n=22) 0.0 Mean Change from Baseline P-value 300mg/2mL 600mg/4mL One-sided
Slide 79: Statistically significant improvement in QMG score at Week 13
Statistically significant improvement in QMG score for both claseprubart arms vs. placebo at Week 13 Mean Change in QMG Score from Baseline at Week 13 Placebo Claseprubart 300mg Q2W Claseprubart 600mg Q2W (n=22) (n=21) (n=22) 0.0 Mean Change from Baseline P-value 300mg/2mL 600mg/4mL -2.0 One-sided P
Slide 80: Statistically significant improvement in MGC at Week 13
Statistically significant improvement in MGC for both claseprubart arms vs. placebo at Week 13 Mean Change in MGC Summed Muscle Score from Mean Change in MGC Score from Baseline at Week 13 Baseline at Week 13 (post-hoc analysis) Claseprubart Claseprubart Claseprubart Claseprubart Placebo 300mg Q2W 6
Slide 81: Statistically significant improvement in MG-QoL-15r for 300mg/2mL
Statistically significant improvement in MG-QoL-15r score for 300mg/2mL vs. placebo at Week 13 Mean Change in MG-QoL-15r Score from Baseline at Week 13 Placebo Claseprubart 300mg Q2W Claseprubart 600mg Q2W (n=22) (n=21) (n=22) 0.0 -1.0 Mean Change from Baseline in MG-QoL-15r Score -2.0 -3.0 -3.9 -4.
Slide 82: Claseprubart demonstrated significant improvements in MG-ADL vs. approved therapies
Claseprubart demonstrated statistically significant and clinically meaningful improvements in MG-ADL Claseprubart S.C. Ultomiris IV Soliris IV Zilbrysq QD PFS Ph II MAGIC Ph III CHAMPION MG Ph III REGAIN Ph III RAISE Week 13 Week 26 Week 26 Week 12 n = 65 n = 78 n = 62 n = 86 BL = 8.4 BL = 9.1 BL =
Slide 83: Additional secondary efficacy measures support best-in-class potential
Additional secondary efficacy measures support claseprubart potential as best-in-class complement inhibitor QMG Score Minimal Symptom Expression (MSE) Claseprubart S.C. Ultomiris IV Claseprubart S.C. Ultomiris IV Ph II MAGIC Ph III CHAMPION MG Ph II MAGIC Ph III CHAMPION MG Week 13 Week 26 Week 13 W
Slide 84: ENJAYMO has no FDA boxed warning and REMS
Unlike C5 inhibitors, ENJAYMO® has no FDA boxed warning and REMS, or prophylactic antibiotic requirement prior to vaccination Antibiotic Prophylaxis Required Boxed Warning and REMS if not Fully Vaccinated C5 inhibitor No Boxed Warning or REMS No Antibiotic Prophylaxis Required C1s inhibitor 84 Sourc
Slide 85: Claseprubart in vitro study indicates lower risk of Neisseria meningitidis infections
Claseprubart in vitro study indicates lower risk of Neisseria meningitidis infections Anti-capsular antibody (Anti -Nm) • Protection against infection is a critical function of the mimics N. meningitidis vaccination 150 complement pathway % Bacterial Survival • DNTH103 selectively inhibits the Class
Slide 86: Claseprubart aims to provide consistent symptom control with Q2W S.C. dosing
Claseprubart aims to provide consistent symptom control with convenient Q2W S.C. dosing • Chronic diseases like MG benefit from consistent treatment and symptom control • When patients are required to take a drug holiday on FcRns, MG-ADL scores immediately begin to rebound as patients self-report sy
Slide 87: Real-world evidence demonstrates clinical advantages for complement vs. FcRns
Real-world evidence demonstrates clinical advantages for complement vs. FcRns Real-world Evidence Indicates Sustained MG-ADL Reductions for Complement Inhibitors vs. FcRns, Among Other Clinical Benefits • Patients on eculizumab showed a greater reduction in MG-ADL over time than those on efgartigimo
Slide 88: Claseprubart improves neurotransmission and muscle contraction in an AChR+ MG model
Claseprubart improves neurotransmission and muscle contraction in an AChR+ MG model Ravulizumab* (1 μM) DNTH103 (0.1 μM) DNTH103 (1 μM) n=3 n=3 n=3 0 Muscle Contraction Fatigue Index • Serum from MG patients used in a validated in vitro (% Change to Baseline, Mean ± SEM) MG model1,2,3 -10 -14.8% • A
Slide 89: Claseprubart restores neuronal conduction velocity in an in vitro CIDP model
Claseprubart restores neuronal conduction velocity in an in vitro CIDP model HV Baseline CV Normalized Conduction Velocity (CV) 1.0 • Serum from 3 CIDP patients was evaluated in a , commercially available in vitro CIDP model1 0.8 (Relative to Baseline) • Assessed improvement in neuronal conduction 0
Slide 90: Claseprubart Ph. 1 healthy volunteer study design
Claseprubart Ph. 1 healthy volunteer study was designed to validate extended half-life, potency and safety SAD SAD 44 HVs enrolled into six cohorts: MAD • Placebo (N= up to 2) I.V. Administration S.C. Administration S.C. Administration • Treated (N= up to 6) SAD 1 (1 mg/kg) MAD 16 HVs enrolled into
Slide 91: Claseprubart has demonstrated deep and sustained complement inhibition in healthy volunteers
Claseprubart has demonstrated deep and sustained complement inhibition in healthy volunteers I.V. SAD: S.C. MAD: PK/PD: Linear PK with Exposure Proportional Strong Accumulation with Q2W Dosing Analysis Demonstrates IC90 of 87 µg/mL Across Doses 1200 150 100 DNTH103 Concentration (µg/mL) 1000 DNTH103
Slide 92: Ph. 1 data estimated IC90 at ~87 µg/mL, supporting target dose
Ph. 1 data estimated IC90 at ~87 µg/mL, leading to target dose of 300mg/2mL in Ph. 2 to achieve steady state >IC90 200 300 Day 0: 15mg/kg I.V. DNTH103 Concentration (µg/mL) Ph. 1 Data Confirms Starting Day 7: • ~60-day half-life 150 300mg S.C. Q2W • IC90 calculated at 87 µg/mL 100 IC90 – 87 µg/mL Do
Slide 93: Claseprubart was generally well tolerated in Ph. 1
Claseprubart was generally well tolerated, with a favorable safety profile in Ph. 1 • No standard safety lab I.V. & S.C. SAD (n=44) S.C. MAD (n=16) findings (hematology, Pooled DNTH103 Pooled DNTH103 Pooled Placebo Pooled Pooled chemistry, coagulation LFTS I.V. S.C. I.V. / S.C. DNTH103 S.C. Placebo
Slide 94: Pro-inflammatory split products C3a and C3b have potential negative effects on NMJ pathology
Pro-inflammatory split products C3a and C3b have potential negative effects on NMJ pathology beyond MAC in gMG C3a and C3b Are Elevated in AChR+ gMG – Both Cause Inflammatory Damage1,2 Active C1s inhibitor Classical pathway claseprubart acts Antibody- upstream from C3 C3a C3b antigen C1q, complex C1
Slide 95: Classical Pathway / aC1s inhibition has the potential to change the CIDP landscape
Classical Pathway / aC1s inhibition has the potential to change the CIDP landscape FcRn Complement Inhibitors Efgartigimod Empasiprubart (C2) Riliprubart (aC1s) Claseprubart (aC1s) S.C. QW I.V. Q4W 600mg/4mL S.C. QW 300mg/2mL S.C. Q2W SoC-Treated SoC Refractory Ph. 3 Study Off Treatment Populations
Slide 96: Broad opportunity for DNTH212 across multiple diseases
Broad opportunity for DNTH212 across multiple diseases where Type 1 interferon and B Cells are implicated Indications with biological rationale and supportive clinical data Indications with biological rationale Biological Biological Rationale Clinical Evidence Rationale Primary Sjögren’s • B Cell: i
Slide 97: Accomplished team of biotech industry veterans and scientists
Accomplished team of biotech industry veterans and scientists committed to bringing innovation to market SENIOR MANAGEMENT BOARD OF DIRECTORS Alison Lawton Chair of the Board Board Member, ProQR and X4, Prior Chair of Board, Magenta Sujay Kango Marino Garcia Simrat Randhawa, M.D. Ryan Savitz Adam Ve
Dianthus Therapeutics — Investor Presentation (August 2026) on 6ix