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Today, Orient EuroPharma (OEP, 4120. TW) has formally announced that it has become the exclusive agent in Taiwan and Southeast Asia markets of Chiesi Global Rare Diseases, a business unit of Chiesi Group for three injectable therapies for the treatment of rare diseases. These injectable treatments are the enzyme replacement therapies (ERT) for three rare conditions: Fabry disease, adenosine deaminase deficiency severe combined immunodeficiency (ADA-SCID), and alpha mannosidosis. ERTs are medical treatment administered to replace a specific enzyme, deficient or absent, in patients affected by specific conditions. These treatments are going to be marketed in Southest Asia region, including Taiwan, Singapore, Malaysia, the Philippines, and Vietnam, offering new therapeutic options to local patients with rare diseases.

• Revcovi® (elapegademase-lvlr) for the treatment of severe combined immunodeficiency due to adenosine deaminase deficiency is one of the few treatment options available for affected patients and has been granted regulatory approval in the United States and Japan.
• Lamzede^® (velmanase alfa) for the treatment of Alpha mannosidosis is the only product approved for the treatment of this indication and has been granted regulatory approval in the EU and some extra-EU Countries (including Brazil).
• PRX-102 under development for the treatment of Fabry disease is the first plant-based, chemically modified, PEGylated enzyme replacement therapy. The mean half-life of the drug is 80 hours, which is significantly longer compared to the currently available enzyme replacement therapies[1]. Results from the latest phase 3 clinical trials in 2022 indicate that treatment with 2 mg/kg of PRX-102 administered by intravenous (IV) infusion every four weeks was well tolerated, and Fabry disease, assessed by estimated glomerular filtration rate (eGFR) slope and plasma lyso-Gb₃ concentration, was stable after 1-year of therapy, in patients previously undergoing long-term treatment with currently available enzymes at the standard, every 2 weeks, administration schedule[2]. PRX-102 is a potential new treatment option for Fabry disease patients, who are currently receiving infusions every two weeks.

Mr. Calvin Tsai, the CEO of OEP, said, “Chiesi is an international pharmaceutical company well-known for its outstanding performance in research and development. With cooperation for 15 years nearly, OEP and Chiesi have built a strong partnership. With the efforts made to explore the markets by OEP, a series of cooperative respiratory medicines are a great success in Taiwan and Southeast Asia markets, which won the affirmation and recognition of Chiesi. OEP has been dedicated to introducing novel and innovative products to the market. As a result, after Chiesi established the Global Rare Diseases Business Unit focusing on the R&D of treatments for rare and extremely rare diseases, OEP proactively took the opportunity to initiate further cooperation in orphan drugs. OEP firmly believes that, with its strategies and marketing channels in Taiwan and Southeast Asia and the novel products manufactured by Chiesi, orphan drugs can be launched rapidly to offer patients better choices. The cooperation between Orient and Chiesi certainly will maximize the advantages of both parties and create a win-win situation.”

“At Chiesi Global Rare Diseases we believe societal attention to address issues impacting millions of people who are affected by a rare disease,” said Giacomo Chiesi, head of Chiesi Global Rare Diseases. “We strive to make our rare disease medicines available to patients around the world and are pleased to partner with OEP to support families impacted by Fabry disease, severe combined immunodeficiency, and alpha mannosidosis in Taiwan and Southeast Asia. We have a long history of commitment to the rare disease community and will continue to reinvest in scientific research and the development of new medications and patient-focused initiatives.”

Taiwan is the fifth market in the world to formulate a specific Act for rare diseases. “The Rare Disease and Orphan Drug Act” has been enforced here for more than two decades. According to the statistics from local Health Promotion Administration, as of January this year, a cumulative number of 19,000 patients suffer from 236 kinds of rare diseases in total in Taiwan. From 2016 to 2020, the special budget allocated to rare diseases increased from NTD 4.5 billion to NTD 7.7 billion, with an increase of approximately 11%. Taking Fabry disease, for example, there are more than 400 patients in Taiwan, and the drug expenses in 2020 exceed NTD 2 billion.

Fabry Disease

Fabry disease is one of the “lysosomal storage disorders.” This disease is caused by a defect in the gene responsible to produce the enzyme α-galactosidase (a-GAL). In the absence of this enzyme, some glycosphingolipids, globotriaosylceramide (GL-3) in particular, cannot be metabolized. Therefore, they accumulate in the cytoplasm and lysosomes of many cells in the body. The accumulation of GL-3 in vascular endothelial cells will cause renal, cardiac, and cerebrovascular lesions. Besides, GL-3 deposits are also found to cause clinically peripheral neuropathy and pain in the extremities.

Severe Combined Immunodeficiency due to Adenosine Deaminase Deficiency (ADA-SCID)

Severe combined immunodeficiency (SCID) due to adenosine deaminase deficiency is a somatic recessive inheritance disorder that compromises the immune system and causes severe combined immunodeficiency (SCID). [3] The cause of the disease is the abnormal accumulation of nucleic acid metabolites caused by the absence of adenosine deaminase (ADA), leading to hypoplasia and dysfunction of T and B lymphocytes. This disease may even result in severe cellular and humoral immune deficiencies.

Alpha - Mannosidosis

Alpha - Mannosidosis is one of the “lysosomal storage disorders.” This disease is caused by a defect in the gene responsible to produce alpha-mannosidase. In the absence of this enzyme, some oligosaccharides cannot be metabolized and thus accumulate in the cytoplasm and lysosomes in many cells in the body. This disease may result in rough facial features, immunodeficiency, polyarticular disease, skeletal weakness, dyskinesia, and impaired intelligence.

About Orient EuroPharma

Founded in 1982, Orient Group was formally listed on Taipei Exchange (4120) in 2003 and has been dedicated to offering a variety of products, such as prescription drugs, cancer medications, nutritional supplements, health products for children and adults, and medical aesthetic products. In addition to the R&D centers established in Taiwan and the United States, OEP is also the owner of two pharmaceutical plants manufacturing oral drug products and injections made of highly sensitizing materials approved by the U.S. FDA. With sales teams across Southeast Asia and the United States, OEP is a multinational pharmaceutical company with strengths in R&D, production, and marketing. The Pharmaceutical Business of OEP aims to become a professional pharmaceutical company specializing in developing and manufacturing products under the 505(b)(2) pathways. In terms of self-development or introduction of new products, OEP has been committed to the development of “new drugs with new values based on the remodeling and optimization of existing technologies,” offering patients better therapeutic options and improving their quality of life. For more information, please visit www.oepgroup.com.tw.

About Chiesi Global Rare Diseases

Chiesi Global Rare Diseases is a business unit of the Chiesi Group established in February 2020 and focused on research and development of treatments for rare and ultra-rare disorders. The Global Rare Diseases unit works in collaboration with Chiesi Group to harness the full resources and capabilities of our global network to bring innovative new treatment options to people living with rare diseases, many of whom have limited or no treatments available. The unit is also a dedicated partner with global leaders in patient advocacy, research and patient care. For more information, please visit www.chiesirarediseases.com.

About Chiesi Group

Chiesi is an international, research-focused biopharmaceuticals group that develops and markets innovative therapeutic solutions in respiratory health, rare diseases, and specialty care. The company’s mission is to improve people’s quality of life and act responsibly towards both the community and the environment.

By changing its legal status to a Benefit Corporation in Italy, the US, and France, Chiesi’s commitment to create shared value for society as a whole is legally binding and central to company-wide decision-making. Since 2019 Chiesi is certified B Corp, meaning that its sustainability efforts are measured and assessed by the most ambitious global standards. The company aims at becoming net-zero by 2035.  

With over 85 years of experience, Chiesi is headquartered in Parma (Italy), operates in 30 countries, and counts more than 6,000 employees. The Group’s research and development centre in Parma works alongside 6 other important R&D hubs in France, the US, Canada, China, the UK, and Sweden.

For further information please visit www.chiesi.com

[1] Schiffmann et al. J Inherit Metab Dis 2019; 42:534

[2] https://protalixbiotherapeutics.gcs-web.com/news-releases/news-release-details/protalix-biotherapeutics-and-chiesi-global-rare-diseases-8