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Lysosomal storage diseases (LSDs) are rare genetic metabolic disorders that cause the accumulation of glycosaminoglycans in lysosomes due to enzyme deficiency or reduced function. Enzyme replacement therapy (ERT) represents the gold standard treatment, but hypersensitivity reaction can occur resulting in treatment discontinuation. Thus, desensitization procedures for different culprit recombinant enzymes can be performed to restore ERT. We searched desensitization procedures performed in LSDs and focused on skin test results, protocols and premedication performed, and breakthrough reactions occurred during infusions. Fifty‐two patients have been subjected to desensitization procedures successfully. Skin tests, with the culprit recombinant enzyme, deemed positive in 29 cases, doubtful in two cases, and not performed in four patients. Moreover, 29 of the 52 desensitization protocols used at the first infusion were breakthrough reaction free. Different desensitization strategies have proved safe and effective in restoring ERT in patients with previous hypersensitivity reactions. Most of these events seem to be Type I hypersensitivity reactions (IgE‐mediated). Standardized in vivo and in vitro testing is necessary to better estimate the risk of the procedure and find the safest individualized desensitization protocol. [ABSTRACT FROM AUTHOR]

This document is a reply to a response to an article titled "Hypersensitivity reaction during enzyme replacement therapy in lysosomal storage disorders: a systematic review of desensitization strategies." The response acknowledges and appreciates the feedback provided by the authors of the response. The article discusses the challenges of managing allergic reactions during enzyme replacement therapy (ERT) for metabolic disorders. It highlights the use of a combined method of rapid desensitization and Allergen Immunotherapy-like (AIT-like) desensitization as a promising strategy. The role of IgG-mediated responses in these reactions is still unclear, and further research is needed. Premedication strategies tailored to the nature of previous reactions are crucial in managing ERT-induced hypersensitivity reactions. The decision to reintroduce ERT after a reaction must consider premedication strategies and the patient's overall health. The document emphasizes the need for ongoing research and evolving strategies to ensure optimal patient outcomes and the efficacy of life-saving therapies. [Extracted from the article]