Abstract Details

Title Hypogammaglobulinemia Independent of Immunotherapy in Individuals with Autoimmune Neurologic Diseases: Insights from a Single Center Case Series

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 1-024

Objective To discuss cases of autoimmune neurologic disease with hypogammaglobulinemia independent of immunotherapy.

Background Autoimmune neurologic diseases are commonly treated with immune modulating medications, some of which may directly (e.g. PLEX) or indirectly (e.g. B cell-depleting therapies) lead to hypogammaglobulinemia. Hypogammaglobulinemia can be identified during initial laboratory screening or while receiving ongoing immunotherapy for the management of these conditions. Identification of hypogammaglobulinemia may suggest underlying immunodeficiency, warranting additional investigation and treatment to prevent infections while also preventing clinical relapse or disease progression.

Design/Methods This is a retrospective case series of individuals seen at the University of Utah Autoimmune Neurology Clinic who met the following criteria: (1) diagnosis of autoimmune neurologic disease and (2) diagnosis of hypogammaglobulinemia (low IgG, IgA and/or IgM) prior to initiation or long after discontinuation of immunotherapy, or that was disproportionate to immunotherapy.

Results Three individuals with 3 distinct autoimmune neurologic diseases and treatment-independent hypogammaglobulinemia were identified: (1) NMDAR encephalitis (NMDARE), (2) MOGAD, and (3) NMOSD. 1/3 individuals had baseline immunoglobulins prior to treatment initiation. All were treated with rituximab during the course of their disease. The individuals with NMDARE and MOGAD were found to have acquired hypogammaglobulinemia during laboratory monitoring following >6 months from last rituximab dose and in the setting of significant infection. The individual with NMOSD was found to have combined variable immunodeficiency in the setting of a VAV1 gene variant, suggesting hereditary immunodeficiency. All experienced long-term control of their autoimmune neurologic conditions without recurrent infections while on IVIG.

Conclusions Hypogammaglobulinemia independent of immunotherapy is a rare condition identified in patients diagnosed with autoimmune neurologic diseases. Clinicians should check quantitative immunoglobulins at baseline to minimize etiologic uncertainty, and monitor levels while treating patients with immunotherapy. Assessing for occult hypogammaglobulinemia and immunodeficiency allows physicians to mitigate associated risks that may be exacerbated while on immunotherapy.

Authors/Disclosures
Jonathan Trout, MD PRESENTER	Dr. Trout has nothing to disclose.
Sydney Lee, MD (University of Utah)	Dr. Lee has nothing to disclose.
Melissa A. Wright, MD (University of Utah)	Dr. Wright has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis .
Ka-Ho Wong (U of U Neurology Clinic)	Mr. Wong has received personal compensation in the range of $0-$499 for serving as a Consultant for CRISPR Therapeutics. The institution of Mr. Wong has received research support from The Sumaira Foundation . The institution of Mr. Wong has received research support from The Siegel Rare Neuroimmune Association. The institution of Mr. Wong has received research support from TG Therapeutics. The institution of Mr. Wong has received research support from Sanofi.
Tammy L. Smith, MD, PhD (Imaging and Neurosciences Center)	Dr. Smith has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. The institution of Dr. Smith has received research support from Alexion/AstraZeneca.
Stacey Clardy, MD, PhD, FAAN (University of Utah)	Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. The institution of Dr. Clardy has received research support from Kyverna. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Advent Health Florida, Beaumont Health, CCF, Emory, Penn State Health, Mayo Clinic, Walter Reed.

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