Joenja Generic Name & Formulations
Mechanism of Action
Leniolisib inhibits PI3K-delta by blocking the active binding site of PI3K-delta. In cell-based assays, leniolisib reduced pAKT pathway activity and inhibited proliferation and activation of B and T cell subsets. Gain-of-function variants in the gene encoding the p110-delta catalytic subunit or loss of function variants in the gene encoding the p85-alpha regulatory subunit each cause hyperactivity of PI3K-delta. Leniolisib inhibits the signalling pathways that lead to increased production of PIP3, hyperactivity of the downstream mTOR/AKT pathway, and to the dysregulation of B and T cells.
Treatment of activated phosphoinositide 3-kinase delta (PI3Kδ) syndrome (APDS).
Joenja Dosage and Administration
Adults and Children
<12yrs: not established. ≥12yrs: (<45kg): not recommended; (≥45kg): 70mg twice daily (~12hrs apart).
Joenja Boxed Warnings
Moderate to severe hepatic impairment: not recommended. Embryo-fetal toxicity. Advise females of reproductive potential to use highly effective contraception during and for 1 week after the last dose. Pregnancy: exclude status prior to initiation. Nursing mothers: not recommended (during and for 1 week after the last dose).
Plasma protein bound: 94.5%.
Fecal (67%), renal (25.5%). Half-life: ~10 hours.
May reduce efficacy of live, attenuated vaccines. Potentiated by strong CYP3A4 inhibitors (eg, itraconazole); avoid concomitant use. Antagonized by moderate and strong CYP3A4 inducers; avoid concomitant use. Avoid concomitant drugs metabolized by CYP1A2 (with a narrow therapeutic index), and substrates of BCRP, OATP1B1, OATP1B3.
Joenja Adverse Reactions
Headache, sinusitis, atopic dermatitis.
Joenja Clinical Trials
Joenja Patient Counseling