Early Intravenous Immunoglobulins May Improve Outcomes in Anti-IgLON5 Disease
Starting intravenous immunoglobulins within the first year of anti-IgLON5 disease onset was associated with better outcomes and fewer deaths over follow-up, according to study results published in JAMA Neurology.
“These findings suggest that immunotherapy should be introduced as early as possible in anti-IgLON5 disease, with intravenous immunoglobulins emerging as a promising treatment option,” wrote first author Thomas Grüter, MD, PhD, of St Josef Hospital, Ruhr-University Bochum, Bochum, Germany, and study coauthors.
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A type of autoimmune encephalopathy, anti-IgLON5 disease often leads to severe disability or death. To gauge whether early immunotherapy is associated with better long-term outcomes, specifically less disability and improved survival, researchers analyzed retrospective data for 107 patients from the German Network for Research on Autoimmune Encephalitis Registry, University Hospital Clinic of Barcelona in Barcelona, Spain, and Erasmus University Medical Center in Rotterdam, Netherlands.
Among the patients, 25 received immunotherapy at the discretion of their treating physicians within the first year of anti-IgLON5 disease onset. Early immunotherapy treatment consisted of intravenous immunoglobulins for 9 patients and rituximab for 13 patients. Another 57 patients received immunotherapy at a later time.
Over a median follow-up of 66 months after disease onset, 44 patients died. Twenty-eight patients died from causes related to anti-IgLON5 disease.
The sole modifiable independent factor linked with a lower incidence of long-term disability and survival was early immunotherapy. According to the study, odds ratios were 0.32 for disability and 2.70 for survival with early immunotherapy.
Despite similar mRS scores at baseline, intravenous immunoglobulin initiated the first year of disease onset was associated with a median modified Rankin Scale (mRS) score of 2 compared with a median mRS score of 3 for other early immunotherapies at the most recent follow-up. Early intravenous immunoglobulin was also linked with fewer deaths: 0 of 9 patients, compared with 6 of 16 patients with other early immunotherapies, analysis showed.
“In this study, early initiation of intravenous immunoglobulins (within the first year of onset) was associated with favorable long-term outcomes and improved survival in anti-IgLON5 disease,” researchers concluded. “Larger prospective studies are needed to validate this finding.”
