AB2 Bio Ltd., a biotechnology company developing innovative therapies for the treatment of severe systemic autoinflammatory diseases and conditions driven by IL-18, have announced the completion of enrolment of its ongoing pivotal Phase 3 study of Tadekinig alfa. The study is designed to show the efficacy and safety of Tadekinig alfa (r-hIL-18BP) for the treatment of primary monogenic IL-18 driven HLH, an ultra-rare and life-threatening condition with no approved therapies, mainly affecting children. AB2 Bio anticipates topline results during second half of 2023.
Dr Djordje Filipovic, CEO of AB2 Bio said:“We are pleased to have completed the targeted enrolment in this pivotal study which represents a significant milestone for AB2 Bio and the patient community. This randomised, placebo-controlled study will provide the dataset required by regulatory authorities to evaluate the efficacy and safety of Tadekinig alfa in patients suffering from primary monogenic IL-18 driven HLH, which will be the basis to support marketing authorisation submissions.”
Primary monogenic IL-18 driven HLH is a potentially life threatening disease characterised by severe systemic inflammation that, if left untreated, may rapidly evolve into multiple-organ failure and death. Mutations in the NLRC4 or XIAP genes are associated with extremely high systemic levels of the pro-inflammatory cytokine IL-18, the therapeutic target of Tadekinig alfa. The excessive release of IL-18 drives the pathology of this clearly defined subgroup of primary HLH, characterized by severe systemic inflammation caused by the detrimental hyperactivation of immune cells leading to a multiorgan pathology and non-reversible organ damage in fatal cases. This ultra-rare disease is most often occurring in infants and young children.
