Neurologic Conditions and Symptoms Reported Among Common Variable Immunodeficiency Patients in the USIDNET
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LETTER TO EDITOR
Neurologic Conditions and Symptoms Reported Among Common Variable Immunodeficiency Patients in the USIDNET Michael Lee 1 Iris M. Otani 2
&
Jenna Nguyen 2 & Ramsay Fuleihan 3 & Katherine Gundling 2 & Charlotte Cunningham-Rundles 4 &
Received: 8 August 2020 / Accepted: 3 September 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abbreviations CVID Common variable immunodeficiency USIDNET United States Immunodeficiency Network IgG-RT Immunoglobulin replacement therapy
To the Editor: Common variable immunodeficiency (CVID) is increasingly recognized as a group of distinct and varied clinical phenotypes associated with a range of inflammatory, autoimmune, and malignant disorders [1]. Neurologic complications in CVID are rare but can be associated with significant morbidity and mortality [2]. Current knowledge regarding neurologic complications in CVID patients is limited to case reports and small case series [2]. To improve our understanding of neurologic complications that occur in CVID, we queried the United States Immunodeficiency Network (USIDNET), a registry of primary immunodeficiency patients, for neurologic conditions and symptoms reported in CVID patient records. We also queried the cohort for age at symptom onset and diagnosis, year of Michael Lee and Jenna Nguyen are the co-first authors Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10875-020-00861-z) contains supplementary material, which is available to authorized users. * Michael Lee [email protected] 1
Department of Medicine, St. Mary’s Medical Center, San Francisco, CA, USA
2
Department of Medicine, Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine, UCSF Medical Center, San Francisco, CA, USA
3
Ann & Robert H Lurie Children’s Hospital of Chicago, Chicago, IL, USA
4
Mount Sinai School of Medicine, New York, NY, USA
birth, race, sex, immunoglobulin replacement therapy (age at initiation of therapy, route, dose), genetic diagnosis, and laboratory data. If multiple immunoglobulin levels were reported, the average was used for analysis. Statistical analyses were performed using Stata/IC 16.0 (StataCorp, College Station, TX). Fischer’s exact test was used for comparisons of proportions, and the Wilcoxon rank-sum test was used to compare medians between two groups. The query returned 1227 CVID patient records. Patients with non-CVID diagnoses were excluded (two with autoimmune lymphoproliferative syndrome; one with BTK mutation), resulting in a cohort of 1224 patients. There were 1315 neurologic conditions and symptoms reported in 49.1% (601/1224) of the USIDNET CVID cohort. Among these 601 patients, the median age of diagnosis was 29.6 years (range 0–73), 65.9% (396/601) were female, and 96.3% (579/ 601) were on immunoglobulin replacement therapy (IgG-RT). Specific gene mutations reported were TACI (n = 25), CD19 (n = 2), ICOS (n = 1), and NFKB2 (n = 4). The distribution of reported neurologic conditions and symptoms among the 6
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