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Delayed Infusion Reactions to Enzyme Replacement Therapies
Zahra Karimian1,2,3, Chester B Whitley4,5,6, Kyle D Rudser7
1Department of Experimental and Clinical Pharmacology, College of Pharmacy, University of Minnesota, Minneapolis, MN, USA. karimian@umn.edu.
JIMD Reports
|August 26, 2016
Summary
Delayed infusion reactions (DIR) in enzyme replacement therapy (ERT) for lysosomal diseases (LD) occur as frequently as concurrent infusion reactions (CIR). Monitoring for both DIR and CIR is crucial for optimizing patient outcomes with ERT.
Area of Science:
- Pharmacology
- Biochemistry
- Clinical Medicine
Background:
- Enzyme replacement therapy (ERT) is available for eight lysosomal diseases (LD), with more products in development.
- Infusion reactions (IR) are common adverse events associated with ERT.
- Infusion reactions are often defined as concurrent infusion reactions (CIR) occurring during ERT, but delayed infusion reactions (DIR) can occur after infusion completion.
Purpose of the Study:
- To evaluate the occurrence of infusion reactions in patients with LD receiving ERT.
- To compare the frequency of ERT-associated infusion reactions to FDA-approved product information.
- To investigate the characteristics and management of both concurrent and delayed infusion reactions.
Main Methods:
- Evaluated infusion reactions in 46 patients with LD receiving ERT for at least two years.
- Assessed reactions based on symptoms, onset time, and duration.
- Compared observed IR frequencies with data in FDA-approved ERT product labeling.
Main Results:
- Delayed infusion reactions (DIR) were observed in the study population.
- DIR occurred as frequently as concurrent infusion reactions (CIR).
- Management strategies for DIR and CIR were found to differ.
Conclusions:
- Delayed infusion reactions (DIR) are a significant concern in ERT for LD, occurring as often as concurrent infusion reactions (CIR).
- Current ERT product labeling often does not characterize or report DIR.
- Effective management of ERT requires monitoring for both DIR and CIR to optimize patient outcomes.
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