Revcovi, Japan’s First Drug for ADA Deficiency, Receives Marketing Approval

Tokyo, Japan --- Teijin Pharma Limited, the core company of the Teijin Group's healthcare business, announced today that it has acquired marketing approval for Revcovi 2.4 mg for intramuscular injection [Elapegademase (genetical recombination)] from the Ministry of Health, Labor and Welfare (MHLW) . Teijin Pharma has exclusive development and marketing rights in Japan to Revcovi, which was developed by Leadiant Biosciences Limited, a UK-based pharmaceuticals company. Revcovi was designated as an orphan drug by MHLW in March 2016.

Adenosine deaminase (ADA) deficiency, an ultra-rare disease caused by a lack of ADA activity due to gene mutations, leads to lymphocyte reduction and severe combined immunodeficiency. It is a designated intractable disease in Japan. The estimated annual incidence of ADA deficiency is between 1/200,000 and 1/1,000,000 live births (Gaspar 2009). Infants with ADA deficiency often die within one year of birth.

The first-line treatment option for ADA deficiency is hematopoietic stem cell transplantation, and other options include ADA enzyme replacement therapy for improvement in immune function and gene therapy. In Japan, however, there remained a high medical need for ADA enzyme replacement therapy, in the situation that no drug for ADA enzyme replacement therapy was approved.

Then, a drug for ADA replacement therapy was publicly offered for development by the Review Committee on Unapproved or Off-Label Drugs with High Medical Needs under MHLW. Teijin Pharma, after successfully applying for the program, concluded an exclusive development and marketing agreement with Leadiant Biosciences Limited (formerly known as Sigma-Tau Pharma Ltd.) in 2014, and then initiated development of Revcovi in Japan.

In a Phase III trial conducted by Teijin Pharma in Japan and a Phase III trial conducted by Leadiant Biosciences, Inc. in the United States, Revcovi injection increased ADA activity and suggested improvement in immune function. Based on these trials, Teijin Pharma submitted its application in June 2018 and has now acquired marketing approval, as announced today.

Revcovi 2.4 mg for intramuscular injection is Japan's first drug for ADA enzyme replacement therapy. It is a recombinant adenosine deaminase analogue chemically modified with polyethylene glycol.

Brand name Revcovi 2.4 mg for intramuscular injection
Nonproprietary name Elapegademase (genetical recombination)
Dosage form Aqueous injection (vial)
Indication Adenosine Deaminase Deficiency
Dosage and Administration The usual intramuscular dose of Revcovi 2.4 mg for Intramuscular injection is Elapegademase (genetical recombination) 0.2 mg/kg of body weight per dose given once a week. The dose should be increased or decreased depending on the patient's conditions. The maximum dose per dose is 0.3 mg/kg. However, if it is necessary to promptly increase the adenosine deaminase activity, it can be injected intramuscularly twice a week at 0.2 mg/kg per dose.

Teijin Pharma will continue to develop drugs and provide new treatment options that contribute to the quality of life for patients with highly unmet medical needs, including rare diseases.

Revcovi is registered trademark of Leadiant Biosciences Limited

Gaspar HB, et al. Blood. 2009; 114: 3524-3532.

About Leadiant Biosciences Limited

Leadiant Biosciences Ltd., based in the United Kingdom, is the European arm of Leadiant Biosciences S.p.A. Formerly known as Sigma-Tau Rare Disease, Leadiant Biosciences is a research-based global pharmaceutical company that dedicates considerable resources to the development, registration and commercialization of novel and effective therapies that address patient needs and improve quality of life.

About the Teijin Group

Teijin (TSE: 3401) is a technology-driven global group offering advanced solutions in the areas of environmental value; safety, security and disaster mitigation; and demographic change and increased health consciousness. Its main fields of operation are high-performance fibers such as aramid, carbon fibers & composites, healthcare, films, resin & plastic processing, polyester fibers, products converting and IT. The group has some 170 companies and around 19,000 employees spread out over 20 countries worldwide. It posted consolidated sales of JPY835 billion (USD 7.6 billion) and total assets of JPY 986.2 billion (USD 9 billion) in the fiscal year ending March 31, 2018.

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