New Ideas. Better Medicines.

39th Annual JP Morgan Healthcare ConferenceJanuary 2021

Forward-Looking Statements

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This presentation contains forward-looking statements that involve substantial risks and uncertainties. In some cases, you canidentify forward-looking statements by the following words: “may,” “will,” “could,” “would,” “should,” “expect,” “intend,” “plan,”“anticipate,” “contemplate,” “believe,” “estimate,” “predict,” “project,” “seek,” “potential,” “continue,” “ongoing” or the negative ofthese terms or other comparable terminology, although not all forward-looking statements contain these words. These statementsrelate to future events or our future financial performance or condition and involve known and unknown risks, uncertainties andother factors that could cause our actual results, levels of activity, performance or achievement to differ materially from thoseexpressed or implied by these forward-looking statements. These risks, uncertainties and other factors are described more fully inour periodic reports filed with the Securities and Exchange Commission (SEC), including our Annual Report on Form 10-K for the yearended December 31, 2019, filed with the SEC on March 10, 2020 and our Form 10-Q filed on November 9, 2020, particularly in thesections titled “Risk Factors” and “Management’s Discussion and Analysis of Financial Condition and Results of Operations”. In lightof the significant uncertainties in our forward-looking statements, you should not place undue reliance on these statements orregard these statements as a representation or warranty by us or any other person that we will achieve our objectives and plans inany specified timeframe, or at all. The forward-looking statements contained in this presentation represent our estimates andassumptions only as of the date of this presentation and, except as required by law, we undertake no obligation to update or revisepublicly any forward-looking statements, whether as a result of new information, future events or otherwise after the date of thispresentation.

This presentation also contains estimates, projections and other information concerning our industry, our business, and the marketsfor our drug candidates, as well as data regarding market research, estimates and forecasts prepared by our management.Information that is based on estimates, forecasts, projections, market research or similar methodologies is inherently subject touncertainties and actual events or circumstances may differ materially from events and circumstances reflected in this information.

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What You Will Hear Today

Vision for the future, following a stellar 2020

Making avacopan a pipeline-in-a-drug

CCX559, a novel approach to checkpoint inhibition

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Looking Back to 2020: A Year of Execution

ü Filed NDA for avacopan (unique inhibitor of complement C5a Receptor, C5aR) in ANCA Vasculitis, accepted with PDUFA target date of July 7, 2021; MAA validated in Europe

ü Announced topline data from three Phase II trials, paving way forward for avacopan in two further orphan indications

ü Identified two new areas to advance to clinical development – avacopan in LN and small molecule immune checkpoint inhibitor in cancer

ü Strengthened balance sheet and scaled up organization

Conclusion Very Strong 2020 Execution is Springboard for 2021

•Our Vision for the Future

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While continuing to reward those who have backed our innovative approach

A fully integrated pharmaceutical company with discovery, development and U.S. commercial capabilities

Changing the treatment paradigm in orphan and rare disease; specifically targeting chronic inflammatory pathway while avoiding immuno-suppression

Launching avacopan for ANCA vasculitis in 2021; potential to address multiple disease areas in the coming years

Continue bringing novel medicines to patients with serious diseases, addressing highest unmet needs

CCXI: Changing the Paradigm with Precision Medicine

§ Chemoattractant receptors attract and activate tissue-damaging inflammatory cells

§ Our highly specific small molecule therapeutics selectively inhibit chemoattractant receptors

§ Precise mechanism of action – precise medicineAnti-inflammatory, not immunosuppressive

§ Our medicinal chemistry expertise now includes developing checkpoint inhibitors for oncology

Extracellular

Intracellular

Cell membrane Receptor

(e.g. C5a Receptor)

Chemoattractant(e.g. C5a)

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All are Small Molecule, Orally-administered Medicines

THERAPEUTIC AREA

Complement Inhibition in Orphan Diseases

IBD and Other Inflammatory and

Autoimmune Diseases

DRUG/TARGET

Avacopan(formerly CCX168)/

C5aR

CCX507/CCR9

CCX587/CCR6

CCX559/ PD-1 /PD-L1

INDICATION PHASE 3 PHASE 2PHASE 1PRECLINICAL

ANCA-ASSOCIATED VASCULITIS

C3 GLOMERULOPATHY

IBD: ULCERATIVE COLITIS

TH17 DRIVEN DISEASE

SEVERE HIDRADENITIS SUPPURATIVA

NDA Under Review

Broad Pipeline from Novel Discovery Platform•

Immuno-Oncology ORALLY ADMINISTERED CHECKPOINT INHIBITORS

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Building Value Through Orphan Diseases

Illustrative Example of Potential Avacopan Annual Sales at Orphan Drug Pricing – U.S. ONLY

~8,000 new cases / year

Prevalence of ANCA vasculitis (both MPA and GPA)

~5,000 new cases / year

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1,000

2,000

3,000

4,000

5,000

6,000

0 5,000 10,000 15,000 20,000 25,000

Gro

ss S

ales

($M

M)

Patients

$200K/Pt/Year

$100K/Pt/Year

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§ Novel medications to treat highest needs

§ Faster pathway to market

§ CCXI fully integrates, retaining U.S. rights

§ Partner provides funding, ex-U.S. sales

§ Multiple potential indications for avacopan

Our Lead Program, Avacopan, is a Perfect Example

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§ Unique selective targeting approach preserves rest of immune system

§ ADVOCATE Phase III pivotal trial demonstrates “better medicine” potential

§ ADVOCATE also showed improved patient life quality with avacopan

§ A home-grown potential blockbuster: multi-billion dollar potential with

avacopan alone

§ Revenue stream to fuel further development

Broadening our Reach

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We continue to develop additional new drug candidatesDiscovery§ Unique, orally-administered small molecules§ Molecular sculpting is a core skill set at CCXI

We are applying our science beyond autoimmune & rare diseaseScope§ Oncology: Plan to start clinical development of orally administered PD-1/PD-L1 inhibitor CCX559 in H1, 2021§ Oncology offers potential fast track for clinical trials, approval and market uptake

We expect to continue to expand our portfolio of new productsPipeline§ Allows us to control our own destiny§ Dynamic nature of pharma industry rewards innovators

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Avacopan

A Novel Orally-Administered Inhibitor of the Complement Fragment C5a Receptor (C5aR)

A Highly Targeted Approach to Complement-Driven Diseases

A ‘Pipeline in a Drug’

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Avacopan

Reason for black box warning for eculizumab

C5aR

C5a Antibodies

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We believe avacopan avoids long-term biological

consequences of ‘upstream’ complement inhibition

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Avacopan does not block C5b-9 production; leaving host defense

mechanism (MAC) in place2

Avacopan preserves beneficial functions of C5L2 pathway3

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Avacopan approach targeting ‘downstream’ complement pathway we believe is best

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Avacopan: Unique Orally Administered C5aR Inhibitor

Avacopan action is here only

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ANCA- Associated Vasculitis: A Complement C5a Activated Neutrophil-Driven Disease

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Highly inflammatory and progressive autoimmune disease

Kidney is a major target organ

Characterized by recurring flares, accruing into irreversible organ system damage (ESRD) and death; relapse is common

Significantly impacts physical function, emotional well-being, and overall productivity

Avacopan ANCA Vasculitis NDA Under Review, PDUFA July 2021; MAA Validated

§ ANCA damage caused by over-activation of complement system; generation of C5a

§ Auto Antibodies →§ Activation of Complement

Cascade →Neutrophils damage blood vessels

§ Avacopan selectively blocks the C5a receptor

§ Prevent activation of disease-causing neutrophils

Rationale for AvacopanCurrent Treatment: Inadequate

Months of high-dose steroids§ Prednisone§ Methylprednisone

Immuno-suppressants§ Cyclophosphamide OR § Rituximab

~40-100K patients in U.S.with ~8.000 diagnosed each year

In-Patient Mortality Rates

In-Patient Serious Infection Rates

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Burden of Hospitalization and Serious Infections in Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis: An Analysis of US National In-Patient Sample Database. ISPOR Value in Health, Vol 23, S34 May 2020

Significant ANCA Inpatient Infection and Mortality Rates

What We Set Out to Prove With Avacopan

Eliminate current therapy- induced illness

Especially from steroid therapy (Measured by GTI2)

Stop accumulated organ damage

From both vasculitis and from steroid therapy toxicity (Measured in part by renal function parameters such as eGFR)

Stop active vasculitis

Reduction in vasculitis activity score (BVAS1, a signs and symptoms index)

Improve patient quality of life

Validated quality of life (QOL) instrumentsPatient reported outcomes (PROs)

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1. Birmingham Vasculitis Activity Score; 2. Glucocorticoid Toxicity Index

A Superior Profile in Treating ANCA Vasculitis

ADVOCATE Demonstrates Avacopan’s Superiority Over Steroid Standard of Care

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Remission at 26 weeks: avacopan 72.3% vs prednisone therapy 70.1%Superior sustained remission at 52 weeks (65.7% vs 54.9%, p=0.0066)✔Stop active vasculitis

without daily prednisone

Risk of Relapse is Significantly Lower with Avacopan Treatment

§ Avacopan (blue line) 10.1% vs prednisone (red) 21.0% (p=0.0091)

§ Avacopan 54% less risk of relapse vs. prednisone group

Pre-specified analysis of relapses at any time after BVAS=0

Hazard ratio of the time to relapse between the two treatment groups is 0.46, 95% CI (0.28, 0.83)

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ADVOCATE Demonstrates Avacopan’s Superiority Over Steroid Standard of Care

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Stop accumulated organ damage

Improve patient quality of life

Remission at 26 weeks: avacopan 72.3% vs prednisone therapy 70.1%Superior sustained remission at 52 weeks (65.7% vs 54.9%, p=0.0066)

Avacopan eliminated the need for daily steroid therapy Reduction in Glucocorticoid Toxicity Index (GTI) and other accepted assessments of glucocorticoid toxicity

Enhanced kidney functionImprovement in estimated glomerular filtration rate

Improved quality of life as assessed by validated patient reported outcome instruments ✔

✔✔✔Stop active vasculitis

without daily prednisone

Reduce current therapy-induced illness

Achieved Statistical Significance Across These Key Measures

Next Steps for Avacopan for ANCA Vasculitis

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NDA under review, PDUFA

July 7, 2021

MAA validated by EMA

JNDA to be filed soon

CCXI Preparing for Commercialization in the U.S.; Vifor Pharma OUS

Planning for U.S. Commercialization in ANCA

§ Rheumatologists and nephrologists primarily manage ANCA vasculitis

§ Newly Diagnosed and Relapsing Patients with Severe Disease primary focus

§ Medical Science Liaisons (MSLs) to perform education and awareness role

§ Specialty Field Force of ~75 to address 80-90% of U.S. market

§ ADVOCATE data reveal avacopan’s considerable value proposition

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KOLs

~ 400 TopPrescribers

~ 3000 Community Specialists

30% of Market

80% of Market

Focused Launch

Avacopan for Hidradenitis Suppurativa (HS)

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Hidradenitis Suppurativa (HS): Debilitating Skin Disease

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1Hoffman et al. PLoS One. 2018;13(9):e0203672

§ Adalimumab is the only approved drug for HS; widely regarded as only having moderate efficacy

§ Still, sales of adalimumab in HS >$1B

§ Precedent for FDA Orphan Drug Designation for moderate or severe HS

HS is a chronic, disabling autoimmune skin disease featuring painful, disfiguring nodules, boils and abscesses

§ HS thought to be a neutrophil-driven disease where C5a involvement is validated (C5aR is elevated in skin biopsies of HS patients1)

§ C5a blockade with avacopan via C5aR has strong potential to control neutrophil activation

§ Oral administration offers advantages over injections (e.g., adalimumab) or infusions

~35-50K patients with severe (Hurley Stage III) HS in the U.S.

Significant Need for New Treatment Options for HS

Current Treatments Rationale for Avacopan

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Phase II AURORA Trial Data in Hurley Stage III HS Patients

§ At 12 weeks, avacopan 30mg BID demonstrated statistically significant improvement in HiSCR vs. placebo

§ Consistent reductions observed in IHS4 and reduction in AN (abscesses and inflammatory nodules), draining fistulae, and abscess count

§ Avacopan was safe and well tolerated

§ Study defined dose, patient population, and safety in HS

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8.9

15.6

2022.2

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12.8

19.1

31.9

42.6*

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0 2 4 6 8 10 12 14

% o

f HiS

CR R

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% HiSCR Hurley Stage III Subgroup

Placebo Avacopan 30 mg BID

∆=20.4; *p=0.0349

Next Step: Phase III Pivotal Trial for Avacopan in Hurley Stage III (Most Severe HS)

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Hurley Stage III HS: Sizeable Market Opportunity

Hurley III is most severe form of HS; ~ 50,000 patients and clear unmet need

Filing for orphan drug designation

Typical orphan drug pricing corridor $100 - $200K per year

$1B+ market opportunity, given reasonable market penetration

Avacopan for C3 Glomerulopathy (C3G)

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C3 Glomerulopathy (C3G): Orphan Kidney Disease with No Approved Therapy

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§ No FDA or EMA approved therapies

§ Non-specific treatment approaches include blood pressure control and broad immunosuppression

§ FDA and EMA Orphan Drug Designations for avacopan

Uncontrolled activation of complement system leading to complement protein deposition in the kidney (glomeruli), disrupting kidney function

§ Characterized by C3, but also C5 / C5a deposition in glomeruli

§ C5a contributes to inflammatory hypercellularity in glomeruli, leading to kidney damage

§ Avacopan targets C5aR, which blocks the effects of C5a

~1,000 – 3,800 patients in U.S.~300-1,000 new cases / year

Discuss Potential Path Forward with FDA Following Positive ACCOLADE Trial Data

Current Treatments Rationale for Avacopan

Primarily affects the young

C3G Histologic Scores for Disease Activity and Chronicity (Renal Biopsy Based)

Placebo

Avacopan

Histology Activity Score(lower score means less acute activity)

26.2

-5.77

-30

-20

-10

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10

20

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40

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60

70

80

Placebo Avacopan

PE

RC

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T C

HA

NG

E F

RO

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AS

ELI

NE

Mean change of disease activity indicates beneficial difference, no statistically significant separation

(due to variability)

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57.52

31.73

0

10

20

30

40

50

60

70

80

Placebo Avacopan

PE

RC

EN

T C

HA

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1.6

0.8

0

0.2

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1.2

1.4

1.6

1.8

2

2.2

Placebo Avacopan

CH

AN

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FR

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BA

SE

LIN

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**P< 0.05

Histology Chronicity Score (lower score means less progression of fibrosis)

Avacopan Reduction of Chronicity Score Provides Evidence for Slowing of Kidney Fibrosis

C3G Histologic Scores Baseline to Week 26, mean ± SEM; Both C5b-9 Strata

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-5

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5

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0 4 8 12 16 20 24 28

% C

hang

e in

eG

FR

Time (weeks)

Placebo Avacopan

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Avacopan Therapy Leads to Significant Improvement in Renal Function in C3G

* P = 0.0221

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-10

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0 4 8 12 16 20 24 28

Perc

ent

Chan

ge f

rom

Bas

elin

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Time (weeks)

Placebo Avacopan

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* P = 0.0199

Improvement from Baseline in eGFR in Patients with eGFR <60 at Baseline

Improvement from Baseline in eGFR in all Patients

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Next up Lupus Nephritis

Renal Function Improvement With Avacopan in C3G is Similar to ADVOCATE Data in ANCA Vasculitis

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Rapid Reduction in Proteinuria in Avacopan Group

Leading to Improvements in eGFR with Avacopan

Proteinuira and eGFR key in other kidney diseases

Potential Expansion: Avacopan in Lupus Nephritis (LN)

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LN - Underserved Progressive Disease with Damage to Kidney Function

§ Poorly controlled with broad immunosuppression, not unlike traditional ANCA Therapy (Glucocorticoids + CYC, etc.)

§ Also mycophenolate, calcineurin inhibitors

§ Kidney transplant not uncommon, does not cure disease

§ Economic burden on HC Systems

Uncontrolled complement system activation implicated in kidney destruction and ESRD in ~20-40% of SLE patients

§ Widely acknowledged need for new, targeted therapy

§ Complement-associated kidney disease

§ Avacopan now demonstrated to improve renal function in ANCA and C3G kidney disease

Prevalence 65,000 – 100,000 in U.S.

Current Treatments Rationale for Avacopan

Primarily affects the young

Predisposition to females

Anders, et al, Nature Reviews 2020 6:7

Small Molecule Checkpoint InhibitorsOrally-Administered Inhibitor for PD-1/PD-L1

CCX559 for the Treatment of Cancer

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Small Molecule Inhibitors for PD-1/PD-L1

Developing proprietary, highly potent, highly specific orally-administered inhibitors of the immune checkpoints PD-L1/ PD-1 for use in human cancer

Small molecule inhibitors of PD-1/PD-L1 interaction offer convenience, dosing flexibility, lower COGS; may better penetrate tumor microenvironment

Considering in combination with chemokine receptor inhibitors that dampen MDSC, with certain antibody therapy regimens, or as monotherapy

CCX559: Plan to Advance into Clinical Development

§ CCX559 − CCX559 is orally bioavailable in all preclinical species; with excellent PK− Predicted human dose of 120 mg QD − Minimal off target effects; clean preclinical safety profile

§ Excellent in vivo anti-tumor activity− Typically better than anti-PD-L1 antibody in pharmacologic models

§ Plan to advance to human studies in 1H 2021

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CCX559 Suppresses Tumor Growth as Well or Better Than Anti-PD-L1 Antibody In Vivo

§ CCX559 dosed orally reproducibly shows clear anti-tumor response– Both by reduced average tumor

volume and increased complete response (CR, i.e., no detectable tumor at time of harvest)

§ CCX559 better than anti-PD-L1 Ab positive control

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**** <0.001, ***<0.01 (2way ANOVA analysis)

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2018 20202019

• Presence of anti-neutrophil cytoplasmic auto-antibodies (ANCA)• Leads to complement system activation and C5a generation• C5a via C5aR is main activator of neutrophils • Neutrophils inflame and destroy blood vessels = vasculitis

History of Capital Efficiency & Strong Financial Position

Financial Highlights

$485.8M cash and investments at September 30, 2020

Excludes $100M credit facility secured in 2020Up to $75M in avacopan regulatory milestones

History of non-dilutive funding fromsuccessful partnering strategy / funding (while retaining 100% US rights)$85M in May 2016 – avacopan$50M in December 2016 – CCX140$20M in February 2017 – avacopan Asia (excl.China)$50M in January 2018 – avacopan CMA validation$21.5M in June 2018 – China rights

End of Quarter Cash and Investments

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•Looking Ahead: Key Catalysts in 2021

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Regulatory Decisions on ANCA Vasculitis

§ NDA in U.S.: PDUFA target date July 7

§ MAA in E.U.§ JNDA in Japan§ Anticipated launch of

avacopan in ANCA

Potential Pathway to Registration in Two More Orphan Diseases

§ Phase III trial of avacopan in severe (Hurley Stage III) HS patients

§ Discuss next steps with FDA regarding avacopan registration in C3G following ACCOLADE

Next Cycle of Pipeline Advancements

CCX559 small molecule checkpoint inhibitor in cancer (H1)

§ Avacopan in Lupus Nephritis (Q3)

Thank You

January 2021