C32-20 A Rare Presentation of Lyme Myocarditis in a Patient With X-Linked Agammaglobulinemia

Abstract Introduction X-linked Agammaglobulinemia (XLA) is a rare primary immunodeficiency that prevents affected individuals from making necessary antibodies to protect them against bacteria, especially those that develop capsules. Lyme Disease (LD), caused by Borrelia burgdorferi, is a relatively common bacterial infection in specific geographic locations that can have a wide variety of presentations from a skin rash to carditis, most commonly heart block but rarely reported as myocarditis as well. There have been no documented associations between this immunodeficiency and LD. Case Report 19-year-old male with PMH of XLA who was admitted to the MICU for shock in setting of fever of unknown origin. He initially presented to the hospital from his infusion clinic two weeks earlier with severe sepsis and a new rash, treated with broad spectrum antibiotics but had a broadly negative infectious workup. He was discharged with plans to follow up with Infectious Disease (ID) outpatient. He re-presented to ED one week after discharge with fluid refractory shock. POCUS revealed a large pericardial effusion and an EF of 25-30% concerning for mixed obstructive and/or cardiogenic shock. He was started back on broad empiric antibiotic coverage. Cardiology performed a right heart catheterization that was interpreted as low-normal SVR and a cardiac index of 3. On hospital day 4, he had a VT/VF arrest. He obtained ROSC but was ultimately placed on VA-ECMO with an impella due to recurrence of VT/VF. An endomyocardial biopsy revealed focal lymphohistiocytic myocarditis interstitial silver positive filamentous forms suspicious for spirochetal infection despite having a negative Lyme antibody earlier in his admission. ID was involved in his care for the duration of his hospital course and once diagnosis was made, he was prescribed a 6-week course of Meropenem and Doxycycline with improvement of his clinical status. Conclusion This case represents a rare presentation of LD where diagnosis was likely delayed due to the patient’s underlying immunodeficiency. LD is typically diagnosed with serum antibody screening tests for both IgG and IgM antibodies to the Borrelia burgdorferi bacteria. As this patient was unable to produce his own gammaglobulins, these tests were falsely negative leading to a delay in diagnosis and treatment. While this immunodeficiency has not been associated with this specific bacteria, it is important to recognize that patients with XLA that present with sepsis should have thorough histories to rule out all potential sources of infection. This abstract is funded by: None