UNITED STATES

SECURITIES AND EXCHANGE COMMISSION

Washington, D.C. 20549

 

Form 6-K

 

Report of Foreign Private Issuer

Pursuant to Rule 13a-16 or 15d-16

under the Securities Exchange Act of 1934

 

September 2019

 

Commission file number: 001-36288

 

Akari Therapeutics, Plc

(Translation of registrant's name into English)

 

75/76 Wimpole Street

London W1G 9RT

United Kingdom

(Address of principal executive offices)

 

Indicate by check mark whether the registrant files or will file annual reports under cover of Form 20-F or Form 40-F.

 

Form 20-F  x       Form 40-F ¨

 

Indicate by check mark if the registrant is submitting the Form 6-K in paper as permitted by Regulations S-T Rule 101(b)(1):_____

 

Indicate by check mark if the registrant is submitting the Form 6-K in paper as permitted by Regulations S-T Rule 101(b)(7):_____

 

 

  

CONTENTS

 

On September 10, 2019, Akari Therapeutics, Plc updated its corporate presentation that it intends to use in conferences and meetings with investors. A copy of the presentation is attached hereto as Exhibit 99.1 and is incorporated herein by reference.

 

Exhibit No.

 

99.1	Corporate presentation dated September 2019.

   

  

SIGNATURES

 

Pursuant to the requirements of the Securities Exchange Act of 1934, the registrant has duly caused this report to be signed on its behalf by the undersigned, thereunto duly authorized.

 

	Akari Therapeutics, Plc
	(Registrant)
	By:	/s/ Clive Richardson
	Name:	Clive Richardson
		Chief Executive Officer and Chief Operating Officer

       

Date: September 10, 2019

 

 

Exhibit 99.1

 

 

September 2019 Investor Presentation Copyright Akari Therapeutics, Plc - 2019

2 Disclaimers Certain statements in this presentation constitute contains “forward - looking” statements within the meaning of Section 27A of th e Securities Act of 1933 and Section 21E of the Securities Exchange Act of 1934. These forward - looking statements reflect our current views about our plans, intentio ns, expectations, strategies and prospects, which are based on the information currently available to us and on assumptions we have made. Although we believe tha t our plans, intentions, expectations, strategies and prospects as reflected in or suggested by those forward - looking statements are reasonable, we can g ive no assurance that the plans, intentions, expectations or strategies will be attained or achieved. Furthermore, actual results may differ materially from t hos e described in the forward - looking statements and will be affected by a variety of risks and factors that are beyond our control. Such risks and uncertainties f or our company include, but are not limited to: needs for additional capital to fund our operations, our ability to continue as a going concern; uncertainties of ca sh flows and inability to meet working capital needs; an inability or delay in obtaining required regulatory approvals for nomacopan (Coversin) and any other produc t c andidates, which may result in unexpected cost expenditures; our ability to obtain orphan drug designation in additional indications; risks inherent in drug de velopment in general; uncertainties in obtaining successful clinical results for nomacopan and any other product candidates and unexpected costs that may result the ref rom; difficulties enrolling patients in our clinical trials; failure to realize any value of nomacopan and any other product candidates developed and being develo ped in light of inherent risks and difficulties involved in successfully bringing product candidates to market; inability to develop new product candidates and sup port existing product candidates; the approval by the FDA and EMA and any other similar foreign regulatory authorities of other competing or superior products brou ght to market; risks resulting from unforeseen side effects; risk that the market for nomacopan may not be as large as expected; risks associated with the depart ure of our former Chief Executive Officers and other executive officers; risks associated with the SEC investigation; inability to obtain, maintain and enforce pa tents and other intellectual property rights or the unexpected costs associated with such enforcement or litigation; inability to obtain and maintain commercial ma nuf acturing arrangements with third party manufacturers or establish commercial scale manufacturing capabilities; the inability to timely source adequate supply of our active pharmaceutical ingredients from third party manufacturers on whom the company depends; unexpected cost increases and pricing pressures and r isk s and other risk factors detailed in our public filings with the U.S. Securities and Exchange Commission, including our most recently filed Annual Rep ort on Form 20 - F filed with the SEC on April 23, 2019. The statements made in this presentation speak only as of the date stated herein, and subsequent events and developments may cau se our expectations and beliefs to change. Unless otherwise required by applicable securities laws, we do not intend, nor do we undertake any obligation, to upd ate or revise any forward - looking statements contained in this presentation to reflect subsequent information, events, results or circumstances or otherwise. W hil e we may elect to update these forward - looking statements publicly at some point in the future, we specifically disclaim any obligation to do so, whether as a result of new information, future events or otherwise, except as required by law. The trademarks included herein are the property of the owners thereof and are used for reference purposes only. Such use shou ld not be construed as an endorsement of such products. This presentation shall not constitute an offer to sell or the solicitation of an offer to buy, nor shall there be any sale o f t hese securities in any state or other jurisdiction in which such offer, solicitation or sale would be unlawful prior to registration or qualification under the sec uri ties laws of any such state or other jurisdiction.

3 Treating Orphan Diseases Caused by Complement & Leukotriene Dysregulation ● Nomacopan (Coversin): Bifunctional inhibitor of C5 & LTB4 Focused on orphan inflammatory & autoimmune diseases where treatment options limited and C5 and LTB4 implicated TMA: Thrombotic Microangiopathy; BP: Bullous Pemphigoid; AKC: Atopic Keratoconjunctivitis; PNH: Paroxysmal Nocturnal Hemoglobinuria ● Potential upcoming value inflection points via multiple mid - stage clinical study readouts in 2019 - 2020 from TMA, BP, and AKC Market Potential Unique MOA Clinical Data Pathway to Approval ● Positive initial clinical readouts across clinical studies ● Excellent safety profile; no drug - related SAEs over 20 cumulative patient - years of cumulative patient treatment data Diversified Targets ● Four orphan conditions currently in Phase II and Phase III studies ‒ Subcutaneous (TMA, BP, & PNH) and topical delivery (AKC) ● $500 million peak sales targets, including follow - on indications ● Platform molecule with a large and active pipeline

4 Dual C5 & LTB4 MOA Provides Unique Option for Multiple Poorly Treated Inflammatory Diseases ● Additive effect demonstrated in preclinical RA, lung and BP models with Nomacopan: significantly more effective than LTB4 or C5 alone ● Elevated levels of C5a and LTB4 seen in human and animal tissue - blister fluid (BP), eye (uveitis), GVHD (HSCT - TMA) ● Nomacopan: good safety profile in clinical studies to date ● Potential to address unmet need across a wide range of conditions ● C5 & LTB4 act synergistically : C5 activates granulocytes, LTB4 promotes granulocyte migration ● Each pathway proven as treatment option: Eculizumab (Soliris ® ) – inhibits C5, and Zileuton ® inhibits LTB4 Background Evidence for Treatment Opportunity ● Clinical response seen across the three target conditions (BP, AKC, HSCT - TMA) where C5 and LTB4 implicated

5 Nomacopan: Well - Designed by Nature Tightly Binding Both C5 & LTB4 Independent, Highly Specific C5 & LTB4 Inhibitory Activity • Nomacopan: biological with high specificity for C5 & LTB4 • Avoids off - target effects by binding ligands not receptors • No immunogenicity to date in human studies • Multiple modes of administration (sub Q, topical, nebulized) • Delivers high degree of patient comfort ‒ Sub Q: Highly soluble & low viscosity ‒ Topical drops: pH neutral / comfortable

6 Nomacopan: Extensive and Growing Positive Efficacy Data Across Four Indications PNH Phase II Achieved primary Phase II end point Six Phase II patients transfusion dependant; two - thirds now transfusion independent 9 patients sub Q ~20 cumulative patient - years* of treatment data TMA BP AKC 2 patients sub Q – chronic Named patient program 3 patients sub Q – chronic ongoing Phase II 3 patients topical – chronic ongoing Phase II PNH 2015 - 2018 2019 Projected Next Step Phase III study naïve patients Initiate pivotal study Q4 2019 Completion of 9 patient Phase II Q4 2019 Completion of 16 patient Phase I/II Q4 2019 Objective to introduce potential pen injector * as of September 2019

7 Subcutaneous Nomacopan: Clinically Testing C5 & LTB4 Binding in HSCT – Thrombotic Microangiopathy

8 HSCT - TMA High Mortality Rate – and No Approved Treatments • Arteriolar thrombi associated with intimal swelling, fibrinoid necrosis, thickening of arterioles and capillaries, and subendothelial accumulation of proteins & cell debris • Cause of TMA after bone marrow transplant not well understood ‒ Can occur in up to 30% patients ‒ Typical diagnostic markers include elevated sC5b9 and proteinuria • Diagnosis & treatment of pediatric TMA - HSCT refined by Dr. Jodele at Cincinnati Children’s Hospital • No approved therapy; current treatment by immunosuppressants • In severe pediatric cases, mortality ≥80% • Orphan condition with a prevalence of circa 5,000 treatable patients

9 Upcoming Pediatric HSCT - TMA Pivotal Trial Potential Expansion to Related Indications • Complement’s role highlighted by elevated terminal complement components (sC5b9) in many patients. • LTB4 believed to have a role in TMA - HSCT in terms of graft vs. host disease (GVHD) progression • Nomacopan used on a named patient basis for pediatric HSCT - TMA • Positive FDA meeting, trial designed around multiple responder endpoints • Nomacopan has received pediatric Fast Track and Orphan status for adult and pediatric HSCT - TMA • Pivotal trial anticipated to commence Q4 2019; primary endpoint TMA response HSCT - TMA Adult TMA - HSCT Other TMA/Vasculitis Pediatric aHUS Rosenthal (2016) Journal of Blood Medicine 7: 181 - 186 Potential expedited approval with a single pivotal study Gateway condition into other TMAs Potential first approval for nomacopan

10 Activity Demonstrated in HSCT – TMA Across Multiple Responder End Points (First 2 Named Patients) TMA Marker Patient Baseline Day 7 Day 14 Day 28 Day 60 Hemolytic anemia 1 Yes Resolved 2 Mild Red blood cell fragments 1 Yes Resolved 2 Yes Resolved Thrombocytopenia 1 Yes Resolved 2 Yes Resolved Increased LDH 1 Yes Resolved 2 Yes Resolved Proteinuria and/or increased creatinine 1 Yes Resolved 2 Yes N/A Hypertension 1 Yes Resolved 2 Yes Resolved Neurology 1 Yes Resolved 2 No GI bleed 1 No 2 Yes Resolved Patient 1 : treated at GOSH made a complete recovery and nomacopan was discontinued after seven weeks Patient 2 : despite resolution of the TMA markers, patient died at day 63 of lung damage considered unrelated to treatment with nomacopan . Note - d ata for patient 2 is up to day 28

11 Nomacopan in HSCT - TMA Differentiation Against Competitors • Patient group to be treated identified via histologic TMA diagnosis in tissue biopsy or laboratory & clinical markers • Daily dosing facilitates more complete complement suppression • LTB4 inhibition may further reduce epithelial activation Clear Positioning Differentiated MOA Next Steps • Pivotal pediatric study scheduled to commence Q4 2019 • Responder - based study based on achieving end points related to disease resolution • Working with FDA on optimizing dosing through the Model - Informed Drug Development (MIDD) Pilot Program

Subcutaneous Nomacopan: Clinically Testing C5 & LTB4 Binding in Bullous Pemphigoid

13 Bullous Pemphigoid Significant Unmet Need • Severe autoimmune blistering skin disorder primarily of the elderly • Caused by immune complex mediated inflammation via autoantibodies • No approved therapy. Current treatment with chronic corticosteroids poses substantial health concerns • KOLs: essentially no change in standard of care in decades • Understanding of BP has grown, but specific therapies still lacking • Orphan condition, with a prevalence of circa 120K patients in US and EU Urticarial - like skin rash Fluid - filled blisters Schematic of dermal - epidermal junction Epidermis Dermis Basement membrane zone Hemi - desmosome Autoantibody targeting leads to separation of the dermis and epidermis BP180 BP230 Key molecular targets that lead to BP pathophysiology Presentation of bullous pemphigoid

14 • Orphan condition in which there is evidence that both terminal complement activation (C5) and LTB4 have a role in driving disease ‒ Preclinical studies with nomacopan showed a dose response and combined C5 & LTB4 more effective than LTB4 alone (1) ‒ Elevated C5/LTB4 in ex - vivo study of BP patients • Clinical objective is steroid sparing and rapid reduction symptoms • Ongoing Phase II study : 42 days – mild to moderate patients Bullous Pemphigoid Evidence of Combined C5 & LTB4 Involvement Bullous Pemphigoid Pemphigus Vulgaris SJS Epidermolysis Bullosa Acquisita Significant market with expansion potential into related conditions 1. Sezin (2019) JCI insight 4: e 128239

15 Prior to treatment, two of three patients were on topical corticosteroids (mometasone), while the third patient was naïve to steroid treatment & received no steroid while on nomacopan. There was no planned tapering of topical steroid dose, but patients did use less than th e 30g permitted to day 21. After day 21, any use of mometasone was regarded as rescue therapy, and both patients were only treated with nomacopan. In the 7 - 11 - day period prior to initiation on nomacopan , the two patients showed either no or minor improvement in BPDAI score (between 0 and 5%), and no improvement in blisters while being treated with topical steroids alone. 0 20 40 0 20 40 60 Day C l i n i c a l S c o r e BPDAI Blisters/Erosions Mean BPDAI and Blister Score First three Patients Phase 2 BP trial Mean BPDAI and Blister Score BPDAI Blisters/Erosions • By Day 7, 21 and day 42 of treatment, the BPDAI global score fell by a mean of 31%, 45% and 52% • By Day 7, 21 and day 42 of treatment, blisters/erosions dropped by a mean of 45%, 75% and 87% Planned amendment to extend into severe patients Daily SQ nomacopan well tolerated, no drug - related adverse events Bullous Pemphigoid Initial Study Data* First 3 Patients: Global and Blisters BPDAI * Nomacopan treatment started

16 Nomacopan in Bullous Pemphigoid Differentiation Against Competitors • Positioned as 2nd line treatment for patients with moderate to severe symptoms, previously treated with corticosteroids • Better safety and side effect profile than steroids and non - specific immunosuppressants • Used to reduce or eliminate dependency on steroids • Nomacopan acts upstream of competitors’ targets – in particular C5 & LTB4 are part of inflammatory process leading to generation of cytokines via antibody Clear Positioning Differentiated MOA Next Steps • Potential post - Phase II regulatory interaction following study completion to help define pivotal study design

17 Topical Nomacopan: Clinically Testing C5 & LTB4 Binding in Ophthalmology / AKC

18 Atopic Keratoconjunctivitis (AKC): Chronic Ocular Disease Which Can Lead to Vision Loss • Chronic disease can result in severe dry eye, corneal neovascularisation, & formation of sterile corneal ulcers • If left untreated, can lead to vision loss; other complications include cataracts, keratoconus, infectious keratitis and tear dysfunction • Topical drugs, such as steroids or cyclosporine treatments complicated by issues with comfort and side effects of chronic usage • Orphan condition with a prevalence of 160K patients in the US and EU Manifestation of AKC • Atopic Keratoconjunctivitis: severe eye surface inflammation causing infiltration of immune cells (neutrophils & T cells)

19 Atopic Keratoconjunctivitis (AKC) AKC VKC PKC SAC SJS MMP • Infiltration of neutrophils & T cells into conjunctival epithelium • LTB4 levels – 20X in allergic conjunctivitis patients • Complement system in tears is functionally active, and concentration of all components is greatly increased in closed - eye tears • C5, likely generated by macrophages, may be aggressively activated by corneal disease (1) • Preclinical model demonstrated equivalence to cyclosporine • Ongoing Phase I/II study. Potential to be first surface of eye biological treatment Different formulation allows potential pricing & licensing flexibility Expansion potential into other poorly - treated surface of the eye diseases 1. Bora (2008) Semin Immunopathol 30: 85 - 95

20 • Patients had deteriorating moderate – severe AKC despite ≥3 months treatment with cyclosporin t.i.d. ± steroids • Part A of study – first in man – successfully completed – drug well tolerated • Overall improvement across both signs and symptoms ‒ Patient 1 had a disease flare at Day 42 but then recovered Initial AKC Clinical Data – First 2 Patients to Day 56 Rapid Improvement in Composite Clinical Scores • No serious drug - related AEs: drops comfortable and well tolerated • Next stage of trial (part B) ongoing ‒ 16 patients randomized, double masked ‒ To enhance recruitment, trial expanded to additional clinical centers 23.3 19.0 16.0 14.5 16.0 10.5 BASELINE 7 14 28 42 DAY 56 Composite Clinical Scores 25 20 15 10 5 0 N=3 3 3 2 2 2

21 Nomacopan in AKC Clear Differentiation Vs. Competitors • Positioned as 1st line treatment for patients with moderate to severe symptoms, given clear unmet need • Comfortable on application (pH neutral) • Rapid improvement in signs & symptoms in human studies • Upstream effects on T cells and granulocytes Clear Positioning Differentiated MOA Next Steps • Potential regulatory interaction following Phase I/II study completion to help define pivotal study design • Opportunity to expand into broader dry eye

22 C5 & LTB4 Activity in Back of the Eye Indications Potential New Target Indication: Uveitis * Eskandarpour M et al. Poster 797 (B0275) ARVO 2019 LTB4 receptor C5a receptor Autoimmune Uveitis (EAU) Model • Intravitreal nomacopan reported significant improvement in uveitis model versus control • Significant down - regulation of Th1/Th17 cells and IL - 17A production • Effect approx. equal to intravitreal dexamethasone • C5 & LTB4 receptors both identified in retina* • Uveitis treatment currently primarily steroids

23 Subcutaneous Nomacopan: Paroxysmal Nocturnal Hemoglobinuria

24 PNH Phase II: Transfusion Reduction Validated Efficacy - Option Value For Continuing to MAA • Six patients transfused in six months prior to entering Phase II • Three became transfusion independent during Phase II • Four transfusion independent during follow - up long - term safety study Percentage Phase II patients transfusion dependent (n=6) at entry who remain transfusion dependent RECEIVING NOMACOPAN % transfusion dependent 0 10 20 30 40 50 60 70 80 90 100 6 months prior to Phase 2 3 months during Phase 2 6 months after Phase 2 in long- term safety study Phase II PNH 67% Decline in Transfusion Dependence • Successful Phase II study achieved primary end point LDH ≤1.8X ULN • Phase III study in naïve patients – primary end point: transfusion independence • Potential pen injector will hold weekly dose, stable at room temperature with 0.4ml daily injection

Wrap up

26 Four Primary/Lead Indications in Phase II/III Plus Pipeline of Earlier - Stage Targets Preclinical Phase I Phase II Phase III * Phase I/II study APS: Antiphospholipid Syndrome; MG: Myasthenia Gravis Ophthalmology AKC* Subcutaneous PNH TMA - HSCT Bullous Pemphigoid Pulmonary Lung (Nebulized form) Engineered MG (LA / Targeted) Uveitis APS

27 ● Growing efficacy data set across four severe orphan conditions ● 20 cumulative patient - years of treatment shows positive safety data and well tolerated ● Multiple expected short - to mid - term clinical readouts: – BP : Complete mild to moderate patients Q4 2019 – TMA : Q4 2019 - pivotal trial approval/open – AKC: Part B update Q4 2019 – PNH: Staged readouts ● $20 million financing facility* with Aspire Capital ● ~1.6 billion ordinary shares outstanding / equivalent to 16 million ADSs Financial and Clinical Wrap up Meaningful Upcoming Clinical Milestones * Circa $5.5 million drawn down to date

APPENDIX

29 ALOX5 Engagement GPCRs BLT1/BLT2 Nomacopan Competitive Advantage: Synergistic C5 and LTB4 inhibition PLA2 Arachidonic acid C3 C3b C3 convertase Opsonisation Amplification loop C5 convertase C3a C5b C6 C7 C9 C8 C5 Engagement GPCRs C5aR/C5aL2 C3b Anaphylatoxins C5a LTB4 Membrane Attack Complex nomacopan LTA4H cysLTs IgG binding to Fc ઻ receptors Cytokines e.g. TNF and IL8 Toll - like receptors C5a/MAC & LTB4 autoactivation IgG and IgM binding to C1q Complex carbohydrates Damage associated molecular patterns INDEPENDENT ACTIVATION PATHWAYS SIGNAL CONTINUALLY IN DEVELOPING & ESTABLISHED DISEASE MEDIATORS OF PATHOLOGY DRIVE DISEASE MAINTENANCE Granulocyte activation Proinflammatory cytokines Procoagulant effects Cell lysis / Proliferation Modulation B and T cells Mast cell degranulation Lipox Interruption of the effector mechanisms expected to quell acquired immune responses that contribute to disease

30 LTB4 Ligand Capture Advantageous and Unique MOA Arachidonic acid LTA 4 Lipoxins LTB 4 LTC 4 LTD 4 LTE 4 5 - LOX FLAP LTA 4 H LTC 4 S Lipoxygenases Pro - inflammatory Anti - inflammatory Spasmogenic Zileuton GSK2190915 DG - 031 MK - 0633 TA - 270 5 - LOX / FLAP inhibitors • Reduces anti - inflammatory lipoxins BLT1/BLT2 antagonists • Realization that anti - inflammatory mediators also signal through BLT1/BLT2 LTA 4 H inhibitors • Secondary anti - inflammatory role for LTA 4 H in degrading pro - inflammatory/ remodelling mediator PGP BLT 1 BLT 2 LY - 293111 ONO - 4057 BIIL284 CP105696 OFF - TARGET EFFECTS OF OTHER INHIBITORS

31 N = 5 mice in each treatment group Start drug treatment Expanding Pipeline Focused on Indications Where C5 and LTB4 Both Involved Zileuton PAS - nomacopan PAS - LTB4 - nomacopan Cell recruitment to Lung Induced by LPS Dual action nomacopan more effective than C5 - only nomacopan (or Zileuton®) alone Dual action C5+LTB4 (PAS - nomacopan ) more effective than inhibition of LTB4 only (PAS - LTB4 - nomacopan ) Work performed in group of Prof Andrew Luster, Mass Gen Hospital, Boston, USA Rheumatoid Arthritis Therapeutic model Vehicle nomacopan Mouse model by Pneumolabs Saturated nomacopan 31

32 LTB4 levels - human blister fluid C5a levels - human blister fluid Elevated levels of LTB4 and C5a indicate activation of complement and synthesis of LTB4 in blisters Epidermolysis Bullosa Acquisita Preclinical Efficacy & Elevated C5a/LTB4 in Bullous Pemphigoid patients Preclinical passive mouse model of epidermolysis bullosa acquisita (EBA) from Dr. Sadik in Lubeck, Germany a leading Bullous Pemphigoid center ● Clear dose response ● ~60% reduction in affected area on nomacopan (SC) compared to vehicle or steroid ● P=0.0023 between vehicle and 250 µg/kg

33 Dual Acting Nomacopan Significantly More Effective Than Long - Acting Nomacopan Only Inhibiting LTB4 ● Long acting LTB4 only nomacopan (10mg/kg) ameliorates blister formation but is less effective than the molar equivalent dose of nomacopan (2.5mg/kg) ● 1mg/kg long acting LTB4 only nomacopan ineffective whereas molar equivalent dose of 0.25mg nomacopan effective (see previous slide) CONCLUSION : Both C5 and LTB4 inhibition needed for full efficacy of nomacopan in EBA model 0 2 4 6 8 10 12 0 1 2 3 4 5 6 7 Day Vehicle treatment 0.1 mg/kg PAS-L-Coversin 1 mg/kg PAS-L-Coversin 10 mg/kg PAS-L-Coversin 2.5 mg/kg Coversin nomacopan nomacopan nomacopan nomacopan ABSA (%)

34 Collaboration with Institute of Ophthalmology, London, UK EIC pre - clinical model of severe eye surface inflammation (Dr Virginia Calder) Significant Benefit Demonstrated in Late - Phase Eye Surface Inflammation • C57/Bl6 mice sensitized to OVA for 14 days • E ye surface challenge d with OVA for 12 days post sensitization (days 0 – 12 ) • Nomacopan and cyclosporin A (CSA) applied once daily on days 4 – 12 after inflammatory response well established • Maximum effect seen after 10 days of nomacopan treatment (late phase of inflammation) • Treatment model of established severe eye surface inflammation • Significant improvement in clinical scores • Histology and cytology data support beneficial role of nomacopan • Reduction in Th9 cells • Reduction in IL - 9

35 Bullous Pemphigoid Ongoing Phase II Study Patients with Mild - to - Moderate Disease Current Study design • Phase II Single arm (n = 9); 42 days treatment • Test role of C5 & LTB4 dual inhibition in improving BP outcomes • Active bullous pemphigoid; newly diagnosed or recurrent Treatment • Nomacopan • Day 1 : 60 mg and 30 mg 12 hours later, Day 2 - 42 : 30 mg od Primary endpoint Safety Secondary endpoints Efficacy evaluated by BPDAI (BP disease activity index) and QoL at day 42 • Trial approved Netherlands (2 sites) and Germany (6 sites) • Amendment planned to treat ≤9 additional moderate - severe patients for ≤ 90 days

36 AKC Phase I/II Proof - of - Principle Trial Design Study design • Phase I/II randomized, double masked , placebo - controlled, safety and efficacy study • Part A : 3 patients open label - safety • Part B : 16 randomized patients • All patients on maximum cyclosporine Treatment • Nomacopan topical eye drops twice daily for 2 months • Placebo eye drops Primary endpoint • Safety, comfort Secondary endpoints • Composite score of clinical signs (6) and symptoms (5)

September 2019 Investor Presentation Copyright Akari Therapeutics, Plc - 2019