liposomal ciclosporin A

Liposomal ciclosporin A (L-CsA) is an inhaled, liposome-encapsulated formulation of the calcineurin inhibitor ciclosporin A, specifically developed for the treatment of bronchiolitis obliterans syndrome (BOS) and chronic lung allograft dysfunction (CLAD) in lung transplant recipients. Developed by Zambon following its acquisition of Breath Therapeutics, the drug is administered via the PARI eFlow high-efficiency nebulizer system. This delivery method is designed to provide high concentrations of the immunosuppressant directly to the small airways, which are the primary site of BOS pathology, while minimizing systemic exposure and the associated risks of nephrotoxicity and neurotoxicity. Ciclosporin A works by binding to the intracellular protein cyclophilin A to form a complex that inhibits calcineurin, a phosphatase required for the dephosphorylation and nuclear translocation of the nuclear factor of activated T-cells (NFAT). This inhibition prevents the transcription of genes encoding pro-inflammatory cytokines, most notably interleukin-2 (IL-2), thereby suppressing T-lymphocyte activation and the subsequent immune-mediated fibroproliferative processes that lead to airway obstruction.

Other names

liposomal cyclosporine Ainhaled liposomal ciclosporin Ainhaled liposomal cyclosporine A