Calliditas Therapeutics AB Stifel Healthcare Conference New York, - PowerPoint PPT Presentation

Calliditas Therapeutics AB Stifel Healthcare Conference New York, November 13, 2018 Renée Aguiar-Lucander, CEO

Disclaimer Important information This presentation may contain certain forward-looking statements and opinions. Forward-looking statements are statements that do not relate to historical facts and events and such statements and opinions pertaining to the future that, by example, contain wording such as “believes”, “estimates”, “anticipates”, “expects”, “assumes”, “forecasts”, “intends”, “could”, “will”, “should”, “would”, “according to estimates”, “is of the opinion”, “may”, “plans”, “potential”, “predicts”, “projects”, “to the knowledge of” or similar expressions, which are intended to identify a statement as forward-looking. This applies, in particular, to statements and opinions in this presentation concerning the future financial returns, plans and expectations with respect to the business and management of Calliditas Therapeutics, future growth and profitability and general economic and regulatory environment and other matters affecting Calliditas Therapeutics. Forward-looking statements are based on current estimates and assumptions made according to the best of Calliditas Therapeutics’ knowledge. Such forward-looking statements are subject to risks, uncertainties, and other factors that could cause the actual results, including Calliditas Therapeutics’ cash flow, financial condition and results of operations, to differ materially from the results, or fail to meet expectations expressly or implicitly assumed or described in those statements or to turn out to be less favorable than the results expressly or implicitly assumed or described in those statements. Accordingly, prospective investors and other third parties should not place undue reliance on the forward-looking statements herein. Calliditas Therapeutics can give no assurance regarding the future accuracy of the opinions set forth herein or as to the actual occurrence of any predicted developments. In light of the risks, uncertainties and assumptions associated with forward-looking statements, it is possible that the future events mentioned in this presentation may not occur. Moreover, the forward-looking estimates and forecasts derived from third-party studies referred to in the presentation may prove to be inaccurate. Actual results, performance or events may differ materially from those in such statements due to, without limitation, changes in general economic conditions, in particular economic conditions in the markets on which Calliditas Therapeutics operates, changes affecting interest rate levels, changes affecting currency exchange rates, changes in competition levels and changes in laws and regulations. The information, opinions and forward-looking statements contained in this announcement speak only as at its date, and are subject to change without notice. 2 Calliditas Therapeutics September 2018

Investment Overview Calliditas 1 Novel treatment of IgA nephropathy (IgAN) with potential disease modifying effect Clear path to market – FDA acceptance of proteinuria as surrogate marker 2 Mode of action targets the origin of the disease – active in the gut 3 Only successful placebo controlled, randomized Ph2b study in IgA nephropathy (150 4 patients) Design of upcoming clinical Phase 3 study NEFIGARD replicates Phase 2b 5 6 Additional potential for pipeline development, in-licensing targeting orphan disease 7 Significant unmet medical need with USD 1bn market opportunity, no approved drugs 3 Calliditas Therapeutics September 2018

Development program is regulatory agreed and de-risked → Clear strategy for the further development and approval of Nefecon from end of Phase Proteinuria – Accepted by FDA as 2b meetings surrogate marker for Phase 3 and accelerated approval… → The first company to receive acceptance by the FDA to use proteinuria as Phase 3 endpoint for approval → Poised to become the first approve drug for broad use in the indication – safe, efficient and convenient. Only successful Phase 2b. …supported with post-approval outcome data based on eGFR → FDA and all major European countries have accepted Phase 3 design and protocol 4 Calliditas Therapeutics September 2018

Clinical advisory board comprising world leading IgAN specialists Global Advisory Board Prof Jonathan Barratt Prof Jürgen Floege Prof Brad Rovin Prof Daniel Cattran Nephrologist Leicester Nephrologist Aachen Nephrologist Ohio Nephrologist University, UK University, Germany State University, USA University of Toronto, Canada SELECTED EXPERIENCE: SELECTED EXPERIENCE: SELECTED EXPERIENCE: SELECTED EXPERIENCE: • On the steering • Executive council • Experienced committee of the member International nephrologist working • Kidney Foundation of International IgA Society of Nephrology closely with Calliditas Canada, PSI, the Nephropathy Network and scientific advisory National Institutes of • Focused on biomarker board of ERA/EDTA Health and the • Involved in many development for Canadian Institutes for clinical trials involving • Responsible for the glomerular diseases Health Research IgA nephropathy STOP-IgAN study Prof Bengt Fellström Prof Richard Lafayette Prof Vladimir Tesar Dr Hernan Trimarchi Nephrologist Nephrologist Nephrologist Nephrologist Akademiska sjukhuset, Stanford University, Charles University in Universidad de Buenos Sweden USA Prague, Czech Republic Aires, Argentina SELECTED EXPERIENCE: SELECTED EXPERIENCE: SELECTED EXPERIENCE: SELECTED EXPERIENCE: • Senior professor at • Editor-in-Chief of ASN • Scientific Advisory • Experienced Department of Kidney News nephrologist Board of ERA/EDTA Medical Sciences and ASN • Member of the • Part of the global IgAN • Inventor of Nefecon Glomerular Disease • Was responsible for steering committee Advisory Committee, the VALIGA study ASN Broad support from key opinion leaders underpin global development program 5 Calliditas Therapeutics September 2018

Our lead indication: IgA nephropathy – large unmet medical need PROFILE ESTIMATED PREVALENCE Genetic predisposition – not sufficient but necessary. Environmental, bacterial, dietary triggers. 130,000-150,000 MAIN MARKET Incidence estimated at 2.5 per 100,000 - For the US market corresponding to approximately 6,000- 7,000 new cases each year 200,000 Normally presents in the 20-30s – more prevalent in men than in women. Up to 50% at risk of ESRD within 10-20 years . POTENTIAL MARKET ~2,100,000 OPPORTUNITIES ~190,000 6 Calliditas Therapeutics September 2018

Implications of current off label approach Recent clinical studies show significant safety issues 1,2 KOL reactions “Until less toxic therapies for IgAN are available, treatment with corticosteroids will need to be made in the context of conflicting evidence, and should likely be limited to patients at highest risk of disease progression who understand the significant risk of adverse events” – Sean Barbour and John Feehally, Curr Opin Nephrol Hypertens 2017 The nephrology community is mindful that infection-related deaths have been observed in recent studies of patients with IgAN treated with corticosteroids, particularly in patients of Asian race with high-risk disease” – Heather Reich, AJKD 2017 7 Calliditas Therapeutics September 2018 Source: 1) Lv et al (2017) JAMA 318(5):432-442. 2) Rauen et al (2015) NEJM 373(23):2225-2236

Disease origin and progression – predominant theory 1 2 3 4 Cluster complexes Peyer´s patches Sugar deficient IgA Immune response IgA nephropathy  Lymphoid tissue;  In patients there is  The sugar-deficient  As the clusters enter  Deposits of immune Peyer’s patches in an increase in a IgA molecules the circulation, they complexes result in the distal part of the subclass of trigger an immune form even larger inflammation, small intestines immunoglobulin response resulting in complexes that necrosis and produces IgA molecules (“IgA”) formation of eventually lodge in destruction of the antibodies which lack a specific antibody clusters the kidneys kidneys’ filtration sugar modification apparatus Source: Suzuki et al, J Am Soc Nephrol 2011;22(10):1795-803; Novak et al, Curr 8 September 2018 Calliditas Therapeutics Opin Nephrol Hypertens 2013; 22(3):287-94; Novak et al, Kidney Dis (Basel). 2015; 1(1):8-18.

Key properties of our lead candidate Nefecon Release profile of Nefecon Comments  Targeted local delivery of potent immunosuppressive agent to Peyer’s patches in the ileum  90% first pass liver metabolism  minimize systemic side effects  Substantially similar design to successful large Phase 2b study  significantly reduced development risk  Unique two-step release profile ─ PH-governed delayed disintegration of the capsule ─ Sustained but fast uptake throughout the Ileum 9 Calliditas Therapeutics September 2018