Calliditas Therapeutics AB

Stockholm, June 2019

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.

2Calliditas Therapeutics March 2019

A specialty pharmaceutical company based in Stockholm, Sweden

Listed on Nasdaq in Sweden in June 2018 – Midcap segment (ticker CALTX)

Market cap June 2019: 180 MUSD

Cash position end Q1, 2019: 63 MUSD

Top 5 Shareholders: Industrifonden, Investinor, B Julander, Gladiator, AFA Insurance

Ongoing Phase 3 read out expected for IgAN in 2H 2020; Pipeline: ODD received for AIH and PBC

3March 2019Calliditas Therapeutics

Investment Summary Calliditas

Novel treatment of IgA nephropathy (IgAN) with potential disease modifying effect

Clear path to market – FDA acceptance of proteinuria as surrogate marker

Mode of action targets the origin of the disease – active in the gut

Only successful placebo controlled, randomized Ph2b study in IgA nephropathy (150 patients)

The ongoing clinical Phase 3 study NEFIGARD replicates Phase 2b

Additional potential for pipeline development, in-licensing targeting orphan disease

Significant unmet medical need with USD 1bn market opportunity, no approved drugs

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4March 2019Calliditas Therapeutics

Our lead indication: IgA nephropathy – large unmet medical need

PROFILE ESTIMATED PREVALENCE Genetic predisposition – required but not sufficient. Environmental, bacterial, dietary triggers.

Normally presents in the 20-30s – more prevalent in men than in women.

130,000-150,000

200,000

~2,100,000

~190,000

MAI

N M

ARKE

TPO

TEN

TIAL

MAR

KET

OPP

ORT

UN

ITIE

S

Up to 50% at risk of ESRD within 10-20 years.

5March 2019Calliditas Therapeutics

Disease origin and progression – predominant theory

March 2019 6Source: Suzuki et al, J Am Soc Nephrol 2011;22(10):1795-803; Novak et al, Curr Opin Nephrol Hypertens 2013; 22(3):287-94; Novak et al, Kidney Dis (Basel). 2015; 1(1):8-18.

Calliditas Therapeutics

IgA nephropathyCluster complexesImmune responseSugar deficient IgAPeyer´s patches1 2 3 4

In patients there is an increase in a subclass of immunoglobulin molecules (“IgA”) which lack a specific sugar modification

The sugar-deficient IgA molecules trigger an immune response resulting in formation of antibody clusters

As the clusters enter the circulation, they form even larger complexes that eventually lodge in the kidneys

Deposits of immune complexes result in inflammation, necrosis and destruction of the kidneys’ filtration apparatus

Lymphoid tissue; Peyer’s patches in the distal part of the small intestines produces IgA antibodies

Nefecon offers local treatment at origin of disease

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Active substance is budesonide

90% first pass liver metabolism minimize systemic side effects

Designed to provide a locally restricted and highly concentrated release of budesonide to the Peyer’s patches for maximum effect

Targeted local delivery of potent immunosuppressive agent to Peyer’s patches in the ileum

Unique two-step release profile

─ PH-governed delayed disintegration of the capsule

─ Pulse like exposure throughout the Ileum

Drug product based on known active ingredient Unique targeted release profile

Bude

soni

de co

ncen

trat

ion

March 2019Company Presentation

Successful Phase 2b trial

March 2019

Primary endpoint: Reduction in proteinuria

2.7%

-27.3%-21.5%

Placebo Nefecon(16 mg)

Nefecon(8 mg)

% ∆

UPC

R

Large study population –150 patients

Randomized, double-blinded, placebo controlled

-9.8%

0.6%

-0.9%

Placebo Nefecon(16 mg)

Nefecon(8 mg)

% ∆

eG

FR

Secondary endpoint:Stabilization of eGFR

Oral dose taken daily over a nine-month period

European study in 62 sites in 10 countries

P (16mg)=0.0026P (8mg) = 0.0064

P (16mg)<0.01P (8mg)<0.03

Only Phase 2b Study in IgAN to reach Primary Endpoint Conclusion – Efficacious and safe drug profile

Efficacy Phase 2b trial of 150 patients demonstrated

clinically relevant reduction in proteinuria and GFR stabilization in the treatment arms

UPCR reduction compared to placebo - 9 months treatment (p=0.0066)

eGFR stabilization compared to placebo – 9 months treatment (p=0.0026)

Safety and tolerability Medication-related adverse effects were

transient and mainly mild (77%) to moderate (22%)

No metabolic adverse events (hypertension, diabetes, weight gain)

No severe infections Benign safety profile of 16 mg Nefecon

8Calliditas Therapeutics

Clinical Phase 3 study NEFIGARD to confirm Phase 2b results

Nefecon Phase 2b design

Run-in

9 months on treatment

Nefecon Phase 2b design

Nefecon Phase 3 design – NEFIGARD

→ Phase 3 study design replicates successful Ph2b

→ 200 versus previous 150 patients (Ph2b)

→ 16mg Nefecon once daily oral dose

→ Surrogate marker; Proteinuria for market approval

Key highlights

9March 2019Calliditas Therapeutics

Endpoint: Proteinuria reduction in IgAN patients

→Around 150 clinical sites in 19 countries

→Up to 450 patients in total

→Read out on first 200 patients basis for approval and US market launch

→Top line read-out estimated H2 2020

→Endpoint: Proteinuria reduction for approval of Phase 3 study…

→…with eGFR in post approval follow up

• Endpoint for Phase 3 study identical to endpoint for Phase 2b: Proteinuria reduction.

• Measured in the first 200 patients after nine months of oral treatment – basis for the accelerated approval in the US / conditional approval in Europe

• Potential for full approval if proteinuria reduction is substantial, or on basis of planned interim analysis in post approval observational study. Data on kidney function expected around 18 months from top line read out

• Convenient, oral medication for broad population with disease modifying potential – avoidance of dialysis

• FPI in November 2018

• Company sponsored statistical framework, Inker et al 2016, basis for FDA surrogate marker acceptance

10Calliditas Therapeutics March 2019

ESRD causes considerable costs to society

March 2019

Cost of dialysis

USD 89,000 USD 70,000-200,000

Average annual cost per person in the US

Hemodialysis treatment in 20131 Based on payer's interviews 20162

Total estimated annual hemodialysis cost in the US of USD 42 billion1

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Cost of transplantation

USD 414,800

Average cost per kidney transplant

Total invoiced fees per transplantation3

Total estimated annual kidney transplantation cost of in the USA of USD 7 billion

Source: 1) U.S. Renal Data System, USRDS 2013 Annual Data Report: Atlas of End-Stage Renal Disease in the United States, National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, 2014. 2) Calliditas estimate based on interviews commissioned by the Company. 3) Milliman, Milliman research report:2017 U.S organ and tissue and transplant cost estimation and discussion.

Calliditas Therapeutics

Commercialization strategy for Nefecon

March 2019

Calliditas believes that IgA nephropathy represents a market opportunity of USD 1bn or more in the US

Rights Maintain all rights to Nefecon in the US in all indications

Commercial strategy

Company managed US commercialization Assess partnerships in EU and RoW Target IgA nephropathy patients at risk of

progressing to ESRD (up to 50%) Earlier stage treatment to prevent

progression and preserve kidney function

Focus on clinics providing treatment of IgAN patients - Hub and spoke structure

Targeting of identified group of nephrologists with a relatively small sales and marketing organization

Collaborate with patient organizations

General commercial strategy US commercialization strategy

Rationale for US commercialization

Significant unmet medical need Desire for early stage and safer treatments Sizeable socioeconomic benefits

Orphan drug Specialist target market Disease modifying potential

12Calliditas Therapeutics

Strong product protection and product exclusivity

March 2019

Orphan designation

Existing patents

Patent strategy

Soft barriers

Granted orphan drug designation in the US and Europe. Data protection and market exclusivity for 7 years and 10 years, respectively

Granted patents covering Nefecon, its formulation and method of manufacturing - runs until 2029

Strategic focus on the US and Europe with extension into other geographical markets

New patent estate initiated in 2018

Significant formulation and manufacturing know-how

Bioequivalence studies would require adaptation

13Calliditas Therapeutics

Pipeline Indications - Autoimmune Hepatitis (AIH)

March 2019Corporate Presentation 14Source: 1) Feld et al, Hepatology 2005;42:53-62, Sahebjam and Vierling, Front Med. 2015 Jun;9(2):187-219. 2) Sahebjam and Vierling, Front Med 2015; 9(2):187-219. 3) Czaja, Expert Opin Drug Saf. 2008;7(3):319-33; Czaja, Liver Int 2009;29(6):816-23; Manns et al, Hepatology 2010;51(6):2193-213 (AASLD 2010 AIH Guideline).

The disease A rare, orphan, chronic inflammation of the liver

The cause is unknown

Leads at variable rates to cirrhosis with complications like portal hypertension, liver failure and liver cancer

Typical symptoms are fatigue, abdominal discomfort, jaundice, enlarged liver, skin rashes, joint pains and in women, loss of menstruation

Standard of care Currently no products approved in the US / care treatment includes immunosuppression with systemic steroids

(prednisone) alone or in combination with azathioprine

Up to 80% of treated patients report steroid related side effects after 2 years and 15% discontinue due to drug related adverse events

Calliditas estimates the intolerance and relapse segments together comprise 35-40% of the total population, or approximately 25,000 patients

Estimated prevalence

50,000 – 80,000

Annual US incidence 0.1 – 1.9

per 100,000

Primary Biliary Cholangitis (PBC)

November 2017Analyst presentation 15Source: 1) Lindor et al, Hepatology. 2009 Jul;50(1):291-308,EASL PBC Clinical Practice Gudielines, Journal of Hepatology 2017; 67:145–172. 2) Nguyen et al, Best Pract Res Clin Gastroenterol 2010; 24(5): 647–654. 3) Kim et al, Gastroenterology 2000;119:1631–1636). 4) EASL PBC Clinical Practice Gudielines, Journal of Hepatology 2017; 67:145–172. 5) Company estimate based on prevalence reported by Kim et al Gastroenterology 2000; 119(6):1631-6. 6) Company estimate based on prevalence reported by Kim et al Gastroenterology 2000; 119(6):1631-6 and Nguyen et al, Best Pract Res Clin Gastroenterol 2010; 24(5):647-54.

The disease A progressive chronic autoimmune disease of the liver

The bile ducts are destroyed by inflammatory processes, bile accumulates in the liver causing an increase in the liver volume (cholestasis)

If untreated, the active liver tissue is destroyed and replaced by fibrous tissue, cirrhosis and liver transplant

Early symptoms include fatigue, itchy skin and dry eyes/mouth. Later stages - liver stiffness, musculoskeletal pain, edema, jaundice and underactive thyroid

Standard of care

Ursodeoxycholic acid (UDCA) and obeticholic acid (Ocaliva) are the only FDA-approved medical treatments for PBC3

No targeted anti-inflammatory therapy is registered in the US or Europe

Previous trials indicates that corticosteroids may alleviate symptoms and improve biochemical and histologic findings4

Estimated prevalence

140,0005

Annual US incidence 0.3 – 5.8

per 100,0002

Out-licensing of Nefecon to Greater China

November 2017Analyst presentation 16

The Market IgA Nephropathy is more common in China than in Europe

or the US, and is cited in publications as being the most common reason for ESRD.

According to a large Chinese epidemiology study1 on over 13,000 renal biopsies, IgAN represented over 30%.

A convenient, oral medication to control the disease with the potential of avoiding ESRD is particularly attractive in a China, taking into account travelling distance to hospitals etc.

The Deal Partnering with Everest Medicines, a well funded, private biopharma company with highly experienced staff

predominantly with big pharma backgrounds. Have a broad pipeline of late stage clinical programs for China

Total deal value of $121m, with $15m in upfront payment

Everest Medicines responsible for the development and commercialization in the territory

Potential to be the first approved medication for IgAN in China, targeting a significant unmet medical need Source: 1) Am J Nephrol 2009;30:268-273

Going forward: focus on Nefecon program & Pipeline

H1 2018

• Filing of new patent application related to Nefecon

Ongoing updates regarding commercial strategy and plans

H2 2018 H1 2019 H2 2019 H1 2020 H2 2020 H1 2021 H1 2022

• NEFIGARD first patient in

• Application for ODD for second indication submitted

• Application for ODD for third indication submitted

• Filing of Pediatric Investigational Plan submitted to EMA

• Approval of ODD designation for second indication

• Approval of ODD designation for third indication

• EMA meeting to discuss surrogate marker

• FDA meeting regarding regulatory pathway for second indication

• 200 patients recruited

• Clinical trial for pipe line indication initiated subject to FDA guidance

• EMA decision regarding pediatric pathway

• Top line read out for 200 patients

• Study fully recruited

• Filing with regulatory agencies for market approval

• Enrolment first patient in treatment modality trials / label expansion

• Interim analysis based on 450 patients for validation of surrogate marker

• Commercial launch of Nefecon

17March 2019Corporate Presentation

Investment Summary Calliditas

Novel treatment of IgA nephropathy (IgAN) with potential disease modifying effect

Clear path to market – FDA acceptance of proteinuria as surrogate marker

Mode of action targets the origin of the disease – active in the gut

Only successful placebo controlled, randomized Ph2b study in IgA nephropathy (150 patients)

The ongoing clinical Phase 3 study NEFIGARD replicates Phase 2b

Additional potential for pipeline development, in-licensing targeting orphan disease

Significant unmet medical need with USD 1bn market opportunity, no approved drugs

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18March 2019Calliditas Therapeutics