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Abstract Details

Demographics, Clinical Characteristics and Neurologic Manifestations of CVID-Associated Granulomatous Disease in a Multi-Institutional Dataset
Autoimmune Neurology
P16 - Poster Session 16 (5:30 PM-6:30 PM)
15-002
To characterize the spectrum of neurologic manifestations seen in CVID-associated granulomatous disease as well as treatments and outcomes in a multi-institutional dataset 
Common variable immunodeficiency (CVID) is a primary immunodeficiency defined by hypogammaglobulinemia after the age of 2-4 years with poor vaccine response in the absence of an alternative explanation for immunodeficiency. A subset of these patients will develop granulomatous disease which may mimic sarcoidosis. Like sarcoidosis, CVID-associated granulomatous disease may affect any organ system including the nervous system. Recognition of this distinct subset of patients has important treatment implications. The spectrum of nervous system complications of CVID-associated granulomatous disease has not been well characterized. 
A retrospective review of patients with biopsy proven granulomatous disease AND CVID or pre-treatment hypogammaglobulinemia who were evaluated by Neurologists at multiple academic institutions
We present a detailed review of cases of CVID-associated granulomatous disease from the University of Utah, the Cleveland Clinic, the University of Colorado, Brigham and Women’s hospital, Vanderbilt University and Johns Hopkins University including demographics, clinical characteristics, neurologic manifestations, treatment and outcomes in our population.  
Conclusion: We present the characterization of a multi-institutional dataset of patients with CVID-associated granulomatous disease.  Failure to identify immunodeficiency prior to initiation of traditional sarcoidosis treatments can be life-threatening in this group of patients. 
Authors/Disclosures
Tammy L. Smith, MD, PhD (Imaging and Neurosciences Center)
PRESENTER 		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.
Shamik Bhattacharyya, MD, FAAN (Brigham and Women's Hospital) Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuroLambda. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Continuum. Dr. Bhattacharyya 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 Wiley. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Merck. The institution of Dr. Bhattacharyya has received research support from Alexion Pharmaceuticals. The institution of Dr. Bhattacharyya has received research support from National Institute of Health. The institution of Dr. Bhattacharyya has received research support from UCB. The institution of Dr. Bhattacharyya has received research support from Genentech. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care.
No disclosure on file
James E. Eaton III, MD (Vanderbilt University) Dr. Eaton has received personal compensation in the range of $0-$499 for serving as a Consultant for Uniqure. Dr. Eaton has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Law Practices. Dr. Eaton has received personal compensation in the range of $0-$499 for serving as a Speaker with Can Do MS.
Jonathan R. Galli, MD (University of Utah) Dr. Galli has nothing to disclose.
Rula Hajj-Ali, MD (Cleveland Clinic) Rula Hajj-Ali, MD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amgen. Rula Hajj-Ali, MD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Zena. Rula Hajj-Ali, MD has received publishing royalties from a publication relating to health care.
Carlos A. Pardo-Villamizar, MD (Johns Hopkins U, Med Dept of Neurology) The institution of Dr. Pardo-Villamizar has received research support from National Institutes of Health. The institution of Dr. Pardo-Villamizar has received research support from Bart McLean Fund for Neuroimmunology Research .
Amanda L. Piquet, MD, FAAN (University of Colorado) The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kyverna . The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech/Roche. The institution of Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. The institution of Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Sands Anderson PC. Dr. Piquet has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Joe Jones Law Firm. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Cortez & Associates. Dr. Piquet has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Falk Waas. The institution of Dr. Piquet has received research support from Rocky Mountain MS Center. The institution of Dr. Piquet has received research support from Roche/Genentech. The institution of Dr. Piquet has received research support from NYU. The institution of Dr. Piquet has received research support from Anokion. The institution of Dr. Piquet has received research support from UCB . The institution of Dr. Piquet has received research support from Foundation for Sarcoidosis. The institution of Dr. Piquet has received research support from Kyverna . Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received publishing royalties from a publication relating to health care. Dr. Piquet has received personal compensation in the range of $10,000-$49,999 for serving as a Litigative Consultant with US-Dept HHS/DICP. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Autoimmune Encephalitis Alliance (AEA) that is relevant to AAN interests or activities. Dr. Piquet has a non-compensated relationship as a Medical Advisory Board Member with Stiff Person Syndrome Research Foundation (SPSRF) that is relevant to AAN interests or activities.
Barney J. Stern, MD, FAAN (Johns Hopkins Outpatient Center) Dr. Stern has received personal compensation in the range of $0-$499 for serving as an officer or member of the Board of Directors for Foundation for Sarcoidosis Research. The institution of Dr. Stern 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 AAN. The institution of Dr. Stern has received research support from NIH/NINDS. The institution of Dr. Stern has received research support from NIH/NINDS. The institution of Dr. Stern has received research support from NIH/NINDS. The institution of Dr. Stern has received research support from NIH/NINDS. Dr. Stern has received publishing royalties from a publication relating to health care.
Jinny O. Tavee, MD (National Jewish Health) Dr. Tavee has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CSL Behring. Dr. Tavee has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Johnson and Johnson. Dr. Tavee has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Dynamed. The institution of Dr. Tavee has received research support from Woolsey. The institution of Dr. Tavee has received research support from Milken Foundation. The institution of Dr. Tavee has received research support from CSL Behring. Dr. Tavee has received personal compensation in the range of $0-$499 for serving as a Article author with Medlink.
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.