Immunodeficiency

Percentage of Chronic Variable Immunodeficiency Patients Who Meet Diagnostic, Evaluation and Treatment Guidelines

2017-2021

Percentage of Chronic Variable Immunodeficiency Patients Who Meet Diagnostic, Evaluation, and Treatment Guidelines

2017-2021

It is recommended that patients with Common Variable Immune Deficiency have their baseline response to multiple vaccines as well as evaluation of B cell subsets checked at the time of diagnosis. ¹ Annual spirometry or a computed tomography (CT) scan of the chest, immunoglobulin G (IgG) level, liver function tests and creatinine levels are also recommended. These guidelines are based on expert panel recommendations formulated through the Immunodeficiency Foundation as well as published recommendations of experts in the field. ², ³ B cell subsets are recommended in the initial lab testing due to recent publications indicating their value in predicting future clinical course. ¹

Statistics and outcome measures were gathered from EPIC and local EMR records for patient visits in 2021. In 2017-2021 100% of patients have been tested for IgG, and immunoglobulin A (IgA) or immunoglobulin M (IgM) levels. Subset testing occurred in 93% of patients in 2017, 86% in 2018, 90% in 2019, 85% in 2020 and 83% in 2021. Vaccination testing was completed in 100% of patients between 2017-2021. Either pulmonary function testing (PFT) or a CT scan of the chest was completed on 97% of patients in 2017, 91% in 2018 and 2019, 80% in 2020 and 90% in 2021. Blood was drawn for liver function tests and creatinine levels in 99% of patients in 2017, and in 100% in 2018 - 2021. Immunoglobulin treatment was administered in 99% of patients between 2017-2019, 91% in 2020 and 94% in 2021. Trough IgG levels were found to be > 600 mg/dL in 100% of patients between 2017 and 2021.

¹Carsetti R, Rosado MM, Donnanno S, et al. The loss of IgM memory B cells correlates with clinical disease in common variable immunodeficiency. J Allergy Clin Immunol. 2005;115(2):412-417.

²Buckley RH. Immune Deficiency Foundation Diagnostic & Clinical Care Guidelines for Primary Immunodeficiency Diseases. IDF Foundation 2015

³Cunningham-Rundles C. How I treat common variable immune deficiency. Blood. 2010 Jul 8;116(1):7-15.

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