Practice Variation in Treatment of NMDAR- IgG Encephalitis Across International Centers from the Clinical Outcomes in Autoimmune Encephalitis Study (COAST)
Nivethitha Manohar1, Tatchaporn Ongphichetmetha2, Meredith Dever1, Nicolas Thompson1, Justin Abbatemarco1, Hesham Abboud3, Adrian Budhram4, Stacey Clardy5, Rajesh Gupta6, Tirisham Gyang7, Eoin Flanagan8, Rodrigo Hasbun6, Julien Hebert9, Jyh Yung Hor10, Sarosh Irani11, Jiraporn Jitprapaikulsan2, Joseph LaPorta12, Soon-Tae Lee13, Ahmad Mahadeen14, Giovanna Manzano15, Marrodan Mariano16, Jennifer McCombe17, Alexandra Muccilli18, Amanda Piquet19, Mayra Montalvo Perero20, Kristine O'Phelan21, John Probasco22, Amy Quek23, Shailee Shah24, Andrew Solomon25, Arun Venkatesan22, Amy Kunchok1
1Cleveland Clinic, 2Mahidol University Siriraj Hospital, 3University Hospitals Cleveland Medical Center, 4Western University, 5University of Utah Health, 6UT Health Houston, 7Ohio State University Wexner Medical Center, 8Mayo Clinic Rochester, 9University Health Network Toronto, 10Penang General Hospital, 11Mayo Clinic Florida, 12University of Cincinnati, 13Seoul National University Hospital, 14University of Mississippi Medical Center, 15University of California Irvine, 16Fleni, 17University of Alberta, 18Unity Health Toronto, 19University of Colorado Anschutz, 20University of Florida, 21University of Miami, 22The Johns Hopkins Hospital, 23National University Hospital, 24Northwestern University, 25University of Vermont
Objective:
To 1) examine the baseline characteristics of patients with NMDAR-IgG encephalitis (NMDAR-IgG AE) and 2) understand practice variation in the treatment of NMDAR-IgG AE across international centers contributing to the Clinical Outcomes in Autoimmune Encephalitis Study (COAST).
Background:
NMDAR-IgG AE is treated with acute immunosuppressive therapy (IST). Second-line therapies like rituximab and cyclophosphamide are used in refractory cases. However, the optimal treatment regimen and duration of treatment is not established.
Design/Methods:
Descriptive statistics summarized baseline cohort characteristics. Outcome measures were modified rankin scale (mRS) and clinical assessment scale for autoimmune encephalitis (CASE). Computations were performed in R, version 4.1.1.
Results:
In this international, multicenter study we included 126 NMDAR-IgG AE patients. The median age at onset was 27 (21, 33) and 98 (77.8 %) were female. Presenting symptoms included psychiatric dysfunction (108 (85.7%)), seizures (81 (64.3%)), and movement disorders (67 (53.2%)). Median baseline mRS was 4 (3,5), and median baseline total CASE score was 9 (6,16). Teratoma was identified in 39/112 (34.8%) patients. CSF showed leukocytosis in 77/118 (65.2%) patients. Median length of stay in the hospital was 39 (17,61) days, and 69/116 (59.5%) patients had an ICU admission for a median of 25 (12,53) days. Median time to first-line IST (IVMP, IVIG, PLEX) was 3 (2,7) weeks after symptom onset. Patients received varied second-line therapies including rituximab (76 (60.3%)), corticosteroids (30 (23.8%)), IV/oral cyclophosphamide (17 (13.5%)), mycophenolate mofetil (8, (6.3%)), azathioprine (6, (4.8%)), tocilizumab (5, (4.0%)), bortezomib (5, (4.0%)), methotrexate (1, (0.8%)) and IVIG (1 (0.8%). At the last follow up visit, patients had a median mRS of 1 (0,2) and median total CASE score of 1 (0,3).
Conclusions:
Multicenter international data demonstrates substantial practice variation across centers, especially regarding the type and duration of second-line therapies. Further studies are underway to determine optimal treatment regimens.
Generative AI Usage
No, did not use generative AI in the drafting or editing in this abstract.
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