Consequences of long-term use of sirolimus

Sirolimus (sirolimus) has been shown to be effective against lymphangioleiomyomatosis (LAM), a rare multisystem tumor disease in women. However, the long-term effects of sirolimus in the treatment of LAM are largely unknown. Sirolimus treatment can effectively improve or stabilize the lung function, oxygen levels, exercise capacity and quality of life of LAM patients for up to 4 years. VEGF-D remained at a low level 4 years after treatment, and sirolimus-related adverse events were minor.

Lymphangioleiomyomatosis(LAM) is a rare multisystem neoplastic disease characterized by cystic lung destruction, angiomyolipomas, and lymphangioleiomyomas. LAM may occur occasionally or in adults with tuberous sclerosis. Cystic remodeling of the lung impairs lung function, leading to progressive dyspnea and ultimately respiratory failure. Sirolimus treatment is well tolerated by patients. LAM patients have been using sirolimus for many years. When sirolimus cannot be used due to insensitivity or resistance to sirolimus, there is an urgent need for new LAM therapies.

SirolimusThe original drug has been launched in China and has entered the scope of Class B medical insurance. SpecificationsThe price of each box of 1mg*10 tablets is about 400 yuan. The domestic drug is only available to patients with anti-rejection reactions in organ transplantation. Patients who do not meet the conditions may not be able to purchase this drug. The original sirolimus drug has also been launched overseas. The price of the Turkish version of Specifications1mg*100 tablets per box is around RMB 2,000 (the price may fluctuate due to exchange rates). The ingredients of the original drugs marketed in China are basically the same as those of foreign original drugs. Currently, there are no generic sirolimus drugs produced in other countries.