From the Research
Lumizyme (alglucosidase alfa) has been associated with transient myocarditis in some patients, and healthcare providers should consider this possibility if symptoms such as chest pain, shortness of breath, or abnormal ECG findings occur during treatment. Myocarditis is an inflammation of the heart muscle that can occur as an adverse reaction to this enzyme replacement therapy used for Pompe disease. The condition is typically transient, meaning it resolves over time, but requires careful monitoring 1. Management includes temporary discontinuation of Lumizyme, supportive care, and possibly anti-inflammatory medications depending on severity. Patients should be evaluated with cardiac biomarkers (troponin, CK-MB), ECG, and echocardiography. Once the myocarditis resolves, Lumizyme may be cautiously reintroduced at a slower infusion rate with premedication using antihistamines, antipyretics, and sometimes corticosteroids to reduce the risk of recurrence 2.
Key Considerations
- The reaction is thought to be immune-mediated, possibly related to antibody formation against the recombinant enzyme or an inflammatory response to the therapy 3.
- Regular cardiac monitoring is recommended for all patients on Lumizyme therapy, especially during the initial treatment period 4.
- The development of anti-alglucosidase antibodies is a known adverse effect of alglucosidase alfa, and patients should be monitored for this complication 5.
- Long-term follow-up results in enzyme replacement therapy for Pompe disease have shown significant prolongation of survival, decrease in cardiomegaly, and improvement in cardiac function and conduction abnormalities 1.
Monitoring and Management
- Patients on Lumizyme therapy should be closely monitored for signs and symptoms of myocarditis, including chest pain, shortness of breath, fatigue, palpitations, or abnormal ECG findings.
- Cardiac biomarkers, ECG, and echocardiography should be used to evaluate patients with suspected myocarditis.
- Temporary discontinuation of Lumizyme and supportive care may be necessary in cases of myocarditis, with cautious reintroduction of the therapy at a slower infusion rate and premedication to reduce the risk of recurrence.