Author Of 2 Presentations
P0709 - Discontinuation of therapy may lead to an increased risk of relapse in patients with neuromyelitis optica even after 5 years of remission (ID 1617)
Abstract
Background
Background: Current treatment guidelines recommend early immunosuppressive therapy (IST) to prevent additional relapses in neuromyelitis optica spectrum disorder (NMOSD). The feasibility of IST discontinuation regarding safety, adverse events, and cost, after long-term remission is achieved is a commonly encountered question in clinical practice.
Objectives
We aimed to evaluate the outcomes of IST discontinuation in patients with anti-aquaporin-4 antibody-positive NMOSD an after a sustained remission period.
Methods
We retrospectively reviewed medical records of 17 patients with anti-aquaporin-4 antibody-positive NMOSD who discontinued IST after a relapse-free period ≥3 years. Anti-aquarporin-4 antibodies were meausred once a year during IST and after IST discontinuation.
Results
IST was discontinued at a median age of 40 years (interquartile range [IQR], 32–51) after a median relapse-free period of 62 months (IQR, 52–73). Among 17 patients, 14 (82%) relapsed after a median duration of 6 months (IQR, 4–34) after IST discontinuation, three (18%) of which had severe attacks: all three patients had a severe attack history before IST. The three patients received steroids followed by plasma exchange for acute treatment, but two showed poor recovery and significant Expanded Disability Status Scale worsening after 6 months of the attack. Six (35%) patients showed seroconversion (from seropositive to seronegative) of anti-aquaporin-4 antibodies at least once during IST, and 5 (29%) were seronegative for anti-aquaporin-4 antibodies at the time of discontinuation. However, 4 (80%) of the 5 patients had seroreversion (from seronegative to seropositive) again after IST discontinuation.
Conclusions
IST discontinuation may increase relapse risk in seropositive NMOSD patients even after 5 years of remission. Given the potential devastating consequence of a single attack of NMOSD, IST discontinuation requires more caution in patients with severe attack prior to IST.
P0722 - Inter-attack astrocyte damage in NMOSD – does it exist? : Longitudinal analysis of serum GFAP (ID 1618)
Abstract
Background
Information on subclinical astrocyte damage can provide further insight into neuromyelitis optica spectrum disorder (NMOSD) pathophysiology and disease-monitoring strategies.
Objectives
To investigate whether astrocyte damage occurs during the inter-attack periods in individuals with NMOSD by longitudinally measurement of serum glial fibrillary acidic protein (sGFAP) at multiple time points.
Methods
sGFAP levels were measured in 187 serum samples from 20 NMOSD patients treated with rituximab (median follow-up: 24 months), and 19 age- and sex-matched healthy controls using a highly sensitive single-molecule array assay. Six NMOSD patients with clinical attacks despite treatment were enrolled for attack-related measurements and the other 14 clinically stable patients were randomly selected from NMOSD cohort of National Cancer Center, Korea.
Results
Significant elevations of sGFAP levels were observed in all clinical attacks, and 95% (19/20) of patients showed reduction of sGFAP levels under the cut-off value (3 standard deviations above the mean concentration of age- and sex-matched healthy controls) within 3 months of their clinical attacks. The sGFAP levels were consistently low during inter-attack periods in 90% (17/19) of patients, whose sGFAP levels once returned to below the cut-off value.
Conclusions
Subclinical astrocyte damage represented by increasing sGFAP levels rarely during inter-attack periods in individuals with NMOSD, while a certain degree of astrocyte damage occurred at the time of clinical attacks without exception and became not evident within 3 months. These results suggest that sGFAP can serve as a reliable biomarker of NMOSD activity.