Assessing neurological disease in patients with common variable immunodeficiency (P5.388)

Neurology(2018)

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摘要
Objective: Describe the demographics and clinical characteristics of patients within the University of Utah Healthcare system with coexistent CVID and neurological disorders. Background: Common variable immunodeficiency (CVID) is defined by hypogammaglobulinemia, poor vaccine response, and onset after the age of two years old in the absence of an alternative explanation for immunodeficiency. Patients are at increased risk of recurrent infections, autoimmunity, and malignancy. There is limited epidemiologic data on the coexistence of CVID and neurological disorders and the relationship between immune dysfunction and neurologic complications remains unclear. Design/Methods: We performed a retrospective review of patients within the University of Utah Healthcare system meeting the following criteria: At least one ICD-9-CM code 279.*or ICD-10-CM code D80.* and D83.* At least one visit with a University of Utah Immunology or Allergy provider At least one visit with a University of Utah Neurology or Ophthalmology provider. Patients not meeting clinical criteria for CVID were excluded. Results: We identified 24 unique patients (18 female, 6 male) meeting the above criteria. Each case is described detailing demographics and clinical data including age of CVID diagnosis, time to intravenous immunoglobulin (IVIG) initiation, pre-IVIG immunoglobulin levels, and characterization of coexisting neurological disease. We also further assessed characteristics of patients with coexisting neuroinfectious and neuroinflammatory disease. Conclusions: Our study reports the occurrence of neurological disease in this CVID population, and highlights the importance of consideration for assessment for CVID and other immunodeficiencies in patients with diagnostically challenging neurologic presentations. Disclosure: Dr. Galli has nothing to disclose. Dr. Williams has nothing to disclose. Dr. Greenlee has received personal compensation in an editorial capacity for Associate Editor for Medlink. Dr. Gundlapalli has nothing to disclose. Dr Clardy has nothing to disclose.
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