Abstract Details

Title One Mechanism, Three Diseases: B-Cell Depletion Tames Neuromyelitis Optica Spectrum Disorder, Systemic Lupus Erythematosus, and Idiopathic Thrombocytopenic Purpura

Topic Autoimmune Neurology

Presentation(s) P7 - Poster Session 7 (8:00 AM-9:00 AM)

Poster/Presentation
Number 2-014

Objective To describe a rare case of coexisting neuromyelitis optica spectrum disorder (NMOSD), systemic lupus erythematosus (SLE), and idiopathic thrombocytopenic purpura (ITP), successfully managed with B-cell depletion therapy (inebilizumab), and systematically review the literature on NMOSD coexistence with other autoimmune diseases (AD)s.

Background NMOSD is an immune-mediated inflammatory disorder targeting aquaporin-4 (AQP4) channels within the central nervous system frequently coexists with systemic ADs with rates ranging from 23-36% across cohorts. The most common associations being autoimmune thyroiditis, SLE, and Sjögren’s syndrome. Four FDA-approved immunotherapies for AQP4-IgG-positive-NMOSD with three mechanisms of action (MOA): B-cell-depletion, complement-inhibition, and interleukin- 6-receptor-blockade. These therapies have approved indications or off-label evidence for managing various ADs, but they may also have safety considerations in specific autoimmune contexts.

Design/Methods Case report and literature review.

Results A 23-year-old female with a history of ITP and SLE presented with headache for 5 days in the context of hypertension and thrombocytopenia. The patient deteriorated due to vasogenic edema of posterior fossa requiring surgical intervention. The etiology of the symptomatic cerebral syndrome was uncertain until AQP4-IgG returned positive at 1:10,000. Initially, the patient was treated with complement-inhibition. Given concerns about potential exacerbation of cutaneous-lupus, treatment was transformed into B-cell-depletion (inebilizumab), enabling a unified therapeutic approach for her three concurrent ADs that has allowed for over three years of disease stability.

Conclusions This case highlights the importance of recognizing NMOSD in patients coexisting with ADs, as delayed recognition can lead to missed diagnosis and lost opportunities of using therapies that address overlapping ADs. Our case shows the efficacy of B-cell-depletion across NMOSD, SLE, and ITP, while also emphasizing that other FDA-approved NMOSD-therapies with distinct MOAs may be potential alternatives depending on the accompanying ADs. These observations emphasize the need for individualized treatment strategies and suggest a shared immunogenetic susceptibility across ADs that warrants further study.

Authors/Disclosures
Suhaib Alquraan, MD PRESENTER	Dr. Alquraan has nothing to disclose.
Ghassab E. Ababneh, MD	Dr. Ababneh has nothing to disclose.
Sam Hooshmand, DO (Medical College of Wisconsin)	Dr. Hooshmand has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genetech USA. Dr. Hooshmand has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for TG Therapeutics . Dr. Hooshmand 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. Hooshmand has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genetech USA. Dr. Hooshmand has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for EMD Serono. Dr. Hooshmand has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for TG Therapeutics . Dr. Hooshmand has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen . The institution of Dr. Hooshmand has received research support from Novartis .

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