NEWS RELEASE
August 25, 2021

MHLW approval of Uptravi® for the Treatment of
inoperable Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
or persistent/recurrent CTEPH after surgery
KYOTO, Japan, August 25, 2021 – Nippon Shinyaku Co., LTD.

(Nippon Shinyaku;Headquarters, Kyoto; President, Toru Nakai) announced that Nippon Shinyaku, which is amarketing authorization holder of Uptravi® Tablets 0.2mg and 0.4mg (Generic name:Selexipag) in Japan, has obtained approval for an additional indication of Chronic
Thromboembolic Pulmonary Hypertension (CTEPH) inoperable or persistent/recurrent
CTEPH after surgery from the Ministry of Health, Labor and Welfare (MHLW).
CTEPH is a disease in which thrombi form inside the pulmonary vessels (pulmonaryembolism), and this causes increasing pressure on the pulmonary arteries and decreasing
blood flow in the lungs and heart. Symptoms include feeling hard to breathe when moving
the body or getting tired quickly. If the disease progresses, other symptoms such as swollen
feet and breathing hard even after a little movement may develop due to poor heart function.
The number of patients in Japan is approximately 4,200, and it is designated as an intractabledisease.
Treatment consists of surgery to remove the thrombus, catheters to widen the blood vessels,
or orally available medicine that opens the pulmonary arteries.
Uptravi® was designated as an orphan drug by MHLW for the indication of CTEPH in June
2016. In Japan, we have developed Uptravi® in collaboration with Janssen Pharmaceutical
K.K. (Janssen Japan; HQs, Tokyo; President, Shuhei Sekiguchi).
We are dedicated to the development and commercialization of therapeutic agents andproviding medical information, focusing on the field of intractable/rare diseases. We hopethat we will be able to contribute to patients suffering from CTEPH, their families, andhealthcare professionals as a result of the approval of this additional indication.

1/2

<About Uptravi®>
Uptravi® is an agonist which binds in a selective and long-acting manner on prostacyclin IPreceptors. Uptravi® shows vasodilatory effect by acting selectively on IP receptor, andimproves pulmonary hemodynamics. In Japan, it was launched in November 2016 for theoral treatment of PAH. Overseas, Janssen, a global collaborator, has received approval forUptravi® in over 60 countries worldwide for the oral treatment of PAH. In Japan, we arepromoting the provision and collection of drug information on Uptravi® in cooperation withJanssen Japan. Uptravi® has also been approved by the U.S. Food and Drug Administration(FDA) for intravenous (IV) treatment of PAH in adult patients with WHO functional class (FC)
II–III, who are temporarily unable to take oral therapy.

<About prostacyclin receptor (IP receptor) agonists>
Binding of PGI2 to prostacyclin (prostaglandin I2/PGI2) receptors in the body causes dilationof peripheral blood vessels and inhibition of platelet aggregation. PGI2 receptor agonists,such as Uptravi®, are drugs that enhance these effects.

<About Orphan Drugs>
This is a medical product designated by the MHLW after reviewing on the fact that the numberof patients in Japan is less than 50,000 and that it is particularly necessary for medical care.

Contact
Corporate Communications Dept., Nippon Shinyaku
FAX: +81-75-321-9128

2/2