Enzyme replacement transformed Pompe disease, but the original therapy reached skeletal muscle inefficiently, limiting benefit for patients with predominantly muscular disease. Nexviazyme (avalglucosidase alfa) was engineered to improve that delivery.
It carries roughly fifteen-fold more mannose-6-phosphate than the earlier enzyme, improving uptake into muscle cells where it clears the glycogen accumulation caused by acid alpha-glucosidase deficiency. Infusions are given every two weeks to patients 1 year and older with late-onset Pompe disease.
Nexviazyme is its developer’s designated successor to alglucosidase alfa and faces no biosimilar competition, but it now competes with an alternative two-component therapy approved later. Converting established patients off the older enzyme is the central commercial dynamic.
