Akebia Reports Third Quarter 2020 Financial Results and Recent Business Updates Presented positive global Phase 3 data for vadadustat from the INNO 2 VATE program for the treatment of anemia due to chronic kidney disease

Akebia Reports Third Quarter 2020 Financial Results and Recent Business Updates

CAMBRIDGE, Mass. November 5, 2020 Akebia Therapeutics, Inc. (Nasdaq: AKBA), a biopharmaceutical company with the purpose of bettering the

lives of people impacted by kidney disease, today reported financial results for the third quarter ended September 30, 2020 and provided business updates, including confirming completion of a pre-NDA

meeting with the U.S. Food and Drug Administration (FDA) for vadadustat. Vadadustat is Akebia s investigational oral hypoxia-inducible factor prolyl hydroxylase inhibitor (HIF-PHI) in development for the

treatment of anemia due to chronic kidney disease (CKD) in adult patients on dialysis and not on dialysis.

We recently completed our pre-NDA meeting with the FDA. This was an important milestone for our vadadustat development program, and we remain on track to submit an NDA to the FDA as early as possible next year. A key component of this NDA is

the positive data from our global Phase 3 INNO2VATE program for the treatment of anemia due to CKD in adult patients on dialysis, which we shared most recently at ASN Kidney Week. These data

were clear and consistent, and showed that vadadustat achieved both the primary and key secondary efficacy endpoints, as well as the primary and key secondary safety endpoints of the program for patients on dialysis. Based on our pre-NDA meeting, we remain confident that these results support the potential approval of vadadustat for the treatment of anemia due to CKD in adult patients on dialysis, said John P. Butler, President and

Chief Executive Officer of Akebia Therapeutics. Subject to regulatory review and approval, we believe vadadustat has the potential to be a new oral standard of care to help address the unmet needs of adult patients on dialysis, including both

incident and prevalent dialysis patients. We believe this could translate into a potential $2 billion market opportunity in the U.S., alone. Together with our collaborator, Otsuka, we look forward to bringing this innovative therapy to patients

on dialysis globally, if approved.

Butler continued, The pre-NDA meeting also allowed us the

opportunity to clarify key questions regarding data from PRO2TECT, our global Phase 3 program for the treatment of anemia due to CKD in adult patients not on dialysis, and we look forward to

working with the FDA in their review of these data. While the PRO2TECT data showed that vadadustat achieved both the primary and key secondary efficacy endpoints, it did not meet the

program s primary safety endpoint for patients not on dialysis, and we remain appropriately cautious in our outlook for potential approval of vadadustat in patients not on dialysis. Importantly, we believe the PRO2TECT data will not adversely impact the potential approvability of vadadustat for the treatment of anemia due to CKD in adult patients on dialysis.

Akebia plans to submit a New Drug Application (NDA) to the FDA for vadadustat as early as possible in 2021

for two indications: (1) the treatment of anemia due to CKD in adult patients on dialysis, and (2) the treatment of anemia due to CKD in adult patients not on dialysis. In addition, Akebia and its collaborator, Otsuka Pharmaceutical Co.

Ltd., are working in close collaboration to prepare a Marketing Authorization Application (MAA) for submission to the European Medicines Agency (EMA) next year.

Recent Business Highlights

Third Quarter Financial Results

Akebia will host a conference call at 9:00 a.m. Eastern Time today, Thursday, November 5th, to discuss its third quarter financial results and recent business

highlights. To listen to the conference call, please dial (877) 458-0977 (domestic) or (484) 653-6724 (international) using conference ID number 2860285. The call will

also be webcast LIVE and can be accessed via the Investors section of the Company s website at http://ir.akebia.com.

conference call will be available two hours after the completion of the call through November 11, 2020. To access the replay, dial (855) 859-2056 (domestic) or (404)

537-3406 (international) and reference conference ID number 2860285. An online archive of the conference call can be accessed via the Investors section of the Company s website at

http://ir.akebia.com.

About Akebia Therapeutics

Akebia Therapeutics, Inc. is a fully integrated biopharmaceutical company with the purpose to better the lives of people impacted by kidney disease. The

Company was founded in 2007 and is headquartered in Cambridge, Massachusetts. For more information, please visit our website at www.akebia.com, which does not form a part of this release.

Vadadustat is an oral hypoxia-inducible

factor prolyl hydroxylase (HIF-PH) inhibitor designed to mimic the physiologic effect of altitude on oxygen availability. At higher altitudes, the body responds to lower oxygen availability with stabilization

of hypoxia-inducible factor, which can lead to increased red blood cell production and improved oxygen delivery to tissues. Vadadustat recently completed its global Phase 3 development program for the treatment of anemia due to CKD. Vadadustat is

not approved by the U.S. Food and Drug Administration (FDA) or any regulatory authority with the exception of Japan s Ministry of Health, Labour and Welfare (MHLW). In Japan, vadadustat is approved as a treatment for anemia due to CKD in both

dialysis-dependent and non-dialysis dependent adult patients.

About Anemia due to Chronic Kidney Disease (CKD)

Anemia is a condition in which a person lacks enough healthy red blood cells to carry adequate oxygen to the body s tissues. It commonly occurs

in people with CKD because their kidneys do not produce enough erythropoietin (EPO), a hormone that helps regulate production of red blood cells. Anemia due to CKD can have a profound impact on a person s quality of life as it can cause

fatigue, dizziness, shortness of breath and cognitive dysfunction. Left untreated, anemia leads to deterioration in health and is associated with increased morbidity and mortality in people with CKD.

IMPORTANT U.S. SAFETY INFORMATION FOR AURYXIA (ferric citrate) CONTRAINDICATION

(ferric citrate) is contraindicated in patients with iron overload syndromes, e.g., hemochromatosis.

WARNINGS AND PRECAUTIONS

Most common adverse reactions with

SPECIFIC POPULATIONS

adverse reactions, contact Akebia Therapeutics at 1-844-445-3799.

Please see full Prescribing Information

Forward-Looking Statements

Statements in this press

release regarding Akebia s strategy, plans, prospects, expectations, beliefs, intentions and goals are forward-looking statements within the meaning of the U.S. Private Securities Litigation Reform Act of 1995, as amended, including but not

limited to statements regarding the potential for obtaining approval of vadadustat in dialysis and the potential for vadadustat to be a new oral standard of care in dialysis; the potential indications for and benefits of vadadustat; submitting

marketing approval of vadadustat, and the timing thereof; the market and clinical opportunity for vadadustat; the belief that the PRO2TECT

data will not adversely impact the potential approvability of vadadustat for the treatment of anemia due to CKD in adult patients on dialysis; the expectation regarding the Company s cash resources; and the timing of our cash runway in relation

to the expected timing of the U.S. launch of vadadustat. The terms believe, confident, expect, look forward, on track, plan, potential, will,

working and similar references are intended to identify forward-looking statements, although not all forward-looking statements contain these identifying words. Each forward-looking statement is subject to risks and uncertainties that

could cause actual results to differ materially from those expressed or implied in such statement, including the timing and content of advice given and decisions made by health authorities, including approval and labeling decisions; the actual time

it takes to make regulatory submissions for vadadustat to health authorities, including the submission of the NDA to the FDA and the submission of the MAA to the EMA; risks associated with the Priority Review Voucher for vadadustat; the potential

direct or indirect impact of the COVID-19 pandemic on our business, operations, and the markets and communities in which we and our partners, collaborators, vendors and customers operate; manufacturing and

quality risks; risks associated with management and key personnel changes and transitional periods; the actual funding required to continue to commercialize our commercial product, to develop and commercialize vadadustat, and to operate the Company;

market acceptance and coverage and reimbursement of our commercial product and vadadustat, if approved; the risks associated with potential generic entrants for our commercial product and vadadustat, if approved; early termination of any of

Akebia s collaborations; Akebia s and its collaborators ability to satisfy their obligations under Akebia s collaboration agreements; the competitive landscape for our commercial product and vadadustat; the scope, timing, and

outcome of any legal, regulatory and administrative proceedings; changes in the economic and financial conditions of the businesses of Akebia and its collaborations partners and vendors; and Akebia s ability to obtain, maintain and enforce

patent and other intellectual property protection for our commercial product, vadadustat and any other product candidates. Other risks and uncertainties include those identified under the heading Risk Factors in Akebia s Quarterly

Report on Form 10-Q for the quarter ended June 30, 2020 and other filings that Akebia may make with the U.S. Securities and Exchange Commission in the future. These forward-looking statements (except as

otherwise noted) speak only as of the date of this press release, and Akebia does not undertake, and specifically disclaims, any obligation to update any forward-looking statements contained in this press release.

Kristen K. Sheppard, Esq.

AKEBIA THERAPEUTICS, INC.

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AKEBIA THERAPEUTICS, INC.

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