Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.

Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.

Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.

Background: s/Aims: Minimally invasive pancreatoduodenectomy (MIPD), such as totally laparoscopic pancreatoduodenectomy (TLPD) or robot-assisted pancreatoduodenectomy (RAPD), is increasingly performed worldwide. This study aimed to compare the perioperative outcomes of TLPD and RAPD, and compare the oncologic outcomes between MIPD and open pancreatoduodenectomy (OPD) for malignant disease. Methods: This retrospective study was conducted at two hospitals that followed similar oncological surgical principles, including the extent of resection. RAPD was performed at Seoul National University Hospital, and TLPD at Seoul National University Bundang Hospital. Patient demographics, perioperative outcomes, and oncological outcomes were analyzed. Propensity score matching (PSM) analysis was performed to compare oncologic outcomes between MIPD and OPD. Results: Between 2015 and 2020, 332 RAPD and 178 TLPD were performed. The rates of Clavian–Dindo grade ≥ 3 complications (19.3% vs. 20.2%, p = 0.816), clinically relevant postoperative pancreatic fistula (9.9% vs. 11.8%, p = 0.647), and open conversions (6.6% vs. 10.5%, p = 0.163) were comparable between the two groups. The mean operation time (341 minutes vs. 414 minutes, p < 0.001) and postoperative hospital stay were shorter in the RAPD group (11 days vs. 14 days, p = 0.034). After PSM, the 5-year overall survival rate was comparable between MIPD and OPD for overall malignant disease (58.4% vs. 55.5%, p = 0.180). Conclusions Both RAPD and TLPD are safe and feasible, and MIPD has clinical outcomes that are comparable to those of OPD. Although RAPD exhibits some advantages, its perioperative outcomes are similar to those associated with TLPD. A surgical method may be selected based on the convenience of surgical movements, medical costs, and operator experience.

Background: s/Aims: Minimally invasive pancreatoduodenectomy (MIPD), such as totally laparoscopic pancreatoduodenectomy (TLPD) or robot-assisted pancreatoduodenectomy (RAPD), is increasingly performed worldwide. This study aimed to compare the perioperative outcomes of TLPD and RAPD, and compare the oncologic outcomes between MIPD and open pancreatoduodenectomy (OPD) for malignant disease. Methods: This retrospective study was conducted at two hospitals that followed similar oncological surgical principles, including the extent of resection. RAPD was performed at Seoul National University Hospital, and TLPD at Seoul National University Bundang Hospital. Patient demographics, perioperative outcomes, and oncological outcomes were analyzed. Propensity score matching (PSM) analysis was performed to compare oncologic outcomes between MIPD and OPD. Results: Between 2015 and 2020, 332 RAPD and 178 TLPD were performed. The rates of Clavian–Dindo grade ≥ 3 complications (19.3% vs. 20.2%, p = 0.816), clinically relevant postoperative pancreatic fistula (9.9% vs. 11.8%, p = 0.647), and open conversions (6.6% vs. 10.5%, p = 0.163) were comparable between the two groups. The mean operation time (341 minutes vs. 414 minutes, p < 0.001) and postoperative hospital stay were shorter in the RAPD group (11 days vs. 14 days, p = 0.034). After PSM, the 5-year overall survival rate was comparable between MIPD and OPD for overall malignant disease (58.4% vs. 55.5%, p = 0.180). Conclusions Both RAPD and TLPD are safe and feasible, and MIPD has clinical outcomes that are comparable to those of OPD. Although RAPD exhibits some advantages, its perioperative outcomes are similar to those associated with TLPD. A surgical method may be selected based on the convenience of surgical movements, medical costs, and operator experience.

Background: s/Aims: Minimally invasive pancreatoduodenectomy (MIPD), such as totally laparoscopic pancreatoduodenectomy (TLPD) or robot-assisted pancreatoduodenectomy (RAPD), is increasingly performed worldwide. This study aimed to compare the perioperative outcomes of TLPD and RAPD, and compare the oncologic outcomes between MIPD and open pancreatoduodenectomy (OPD) for malignant disease. Methods: This retrospective study was conducted at two hospitals that followed similar oncological surgical principles, including the extent of resection. RAPD was performed at Seoul National University Hospital, and TLPD at Seoul National University Bundang Hospital. Patient demographics, perioperative outcomes, and oncological outcomes were analyzed. Propensity score matching (PSM) analysis was performed to compare oncologic outcomes between MIPD and OPD. Results: Between 2015 and 2020, 332 RAPD and 178 TLPD were performed. The rates of Clavian–Dindo grade ≥ 3 complications (19.3% vs. 20.2%, p = 0.816), clinically relevant postoperative pancreatic fistula (9.9% vs. 11.8%, p = 0.647), and open conversions (6.6% vs. 10.5%, p = 0.163) were comparable between the two groups. The mean operation time (341 minutes vs. 414 minutes, p < 0.001) and postoperative hospital stay were shorter in the RAPD group (11 days vs. 14 days, p = 0.034). After PSM, the 5-year overall survival rate was comparable between MIPD and OPD for overall malignant disease (58.4% vs. 55.5%, p = 0.180). Conclusions Both RAPD and TLPD are safe and feasible, and MIPD has clinical outcomes that are comparable to those of OPD. Although RAPD exhibits some advantages, its perioperative outcomes are similar to those associated with TLPD. A surgical method may be selected based on the convenience of surgical movements, medical costs, and operator experience.

Background: and Purpose Unlike other immune-mediated neuropathies, anti-myelin-associated glycoprotein (MAG) neuropathy is often refractory to immunotherapy. It is necessary to compare the relative efficacies of various immunotherapies and develop objective biomarkers in order to optimize its clinical management. Methods: This study recruited 91 patients with high anti-MAG antibody titers from 7 tertiary hospitals in South Korea. We analyzed the baseline characteristics, therapeutic outcomes, and nerve conduction study (NCS) findings of 68 patients and excluded 23 false positive cases. Results: The rate of positive responses to treatment was highest using zanubrutinib (50%) and rituximab (36.4%), followed by corticosteroids (16.7%), immunosuppressants (9.5%), intravenous immunoglobulin (5%), and plasma exchange (0%). Disability and weakness were significantly associated with multiple NCS parameters at the time of diagnosis, especially distal compound muscle action potential (CMAP) amplitudes. Moreover, the longitudinal trajectory of the average CMAP amplitudes paralleled the clinical courses, with a 16.2 percentile decrease as an optimal cutoff for predicting a clinical exacerbation (area under the receiver operating characteristic curve=0.792). Conclusions Our study supports the use of NCS as an objective marker for estimating disease burden and tracking clinical changes in patients with anti-MAG neuropathy. We have described the beneficial effects of rituximab and a new drug, zanubrutinib, compared with conventional immunotherapies.