Carolin Schwake, Theodoros Ladopoulos, Vivien Häußler, Ingo Kleiter, Marius Ringelstein, Orhan Aktas, Tania Kümpfel, Daniel Engels, Joachim Havla, Martin W Hümmert, Julian Reza Kretschmer, Daria Tkachenko, Corinna Trebst, Ana Beatriz Ayroza Galvão Ribeiro Gomes, Anne-Katrin Pröbstel, Mirjam Korporal-Kuhnke, Brigitte Wildemann, Sven Jarius, Refik Pul, Mosche Pompsch, Markus Krämer, Florian Then Bergh, Clemens Gödel, Patricia Schwarz, Markus C Kowarik, Paulus Stefan Rommer, Ioannis Vardakas, Makbule Senel, Alexander Winkelmann, Nele Retzlaff, Martin S Weber, Leila Husseini, Annette Walter, Patrick Schindler, Judith Bellmann-Strobl, Friedemann Paul, Ralf Gold, Ilya Ayzenberg
{"title":"多发性骨髓增生异常综合征(MOGAD)的血液透析疗法:对 571 次发作中 117 次治疗干预的回顾性研究。","authors":"Carolin Schwake, Theodoros Ladopoulos, Vivien Häußler, Ingo Kleiter, Marius Ringelstein, Orhan Aktas, Tania Kümpfel, Daniel Engels, Joachim Havla, Martin W Hümmert, Julian Reza Kretschmer, Daria Tkachenko, Corinna Trebst, Ana Beatriz Ayroza Galvão Ribeiro Gomes, Anne-Katrin Pröbstel, Mirjam Korporal-Kuhnke, Brigitte Wildemann, Sven Jarius, Refik Pul, Mosche Pompsch, Markus Krämer, Florian Then Bergh, Clemens Gödel, Patricia Schwarz, Markus C Kowarik, Paulus Stefan Rommer, Ioannis Vardakas, Makbule Senel, Alexander Winkelmann, Nele Retzlaff, Martin S Weber, Leila Husseini, Annette Walter, Patrick Schindler, Judith Bellmann-Strobl, Friedemann Paul, Ralf Gold, Ilya Ayzenberg","doi":"10.1136/jnnp-2024-334863","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Incomplete attack remission is the main cause of disability in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Apheresis therapies such as plasma exchange and immunoadsorption are widely used in neuroimmunology. Data on apheresis outcomes in MOGAD attacks remain limited.</p><p><strong>Methods: </strong>We retrospectively evaluated all apheresis treated attacks occurring in patients with MOGAD between 2008 and 2023 at 18 Neuromyelitis Optica Study Group centres. Treatment response was categorised as complete, partial or no remission. Preattack and follow-up Expanded Disability Status Scale (EDSS) and visual Functional System Scores (FSS) were used to calculate absolute outcomes (ΔEDSS/Δvisual FSS). Predictors of complete remission were analysed using a generalised linear mixed model.</p><p><strong>Results: </strong>Apheresis was used for 117/571 (20.5%) attacks in 85/209 (40.7%) patients. Attacks with simultaneous optic neuritis and myelitis were treated more often with apheresis (42.4%, n=14) than isolated myelitis (25.2%, n=35), cerebral manifestation (21.0%, n=17) or isolated optic neuritis (17.6%, n=51). Apheresis was initiated as first-line therapy in 12% (4.5 (IQR 0-11) days after attack onset), second-line therapy in 62% (15 (IQR 6.75-31) days) and third-line therapy in 26% (30 (IQR 19-42) days). Complete remission was achieved in 21%, partial remission in 70% and no remission in 9% of patients. First-line apheresis (OR 2.5, p=0.040) and concomitant disease-modifying therapy (OR 1.5, p=0.011) were associated with complete remission. Both parameters were also associated with a favourable ΔEDSS. No differences in outcomes were observed between the apheresis types.</p><p><strong>Conclusion: </strong>Apheresis is frequently used in MOGAD attacks. An early start as first-line therapy and concomitant disease-modifying therapy predict full attack recovery.</p>","PeriodicalId":16418,"journal":{"name":"Journal of Neurology, Neurosurgery, and Psychiatry","volume":null,"pages":null},"PeriodicalIF":8.7000,"publicationDate":"2024-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Apheresis therapies in MOGAD: a retrospective study of 117 therapeutic interventions in 571 attacks.\",\"authors\":\"Carolin Schwake, Theodoros Ladopoulos, Vivien Häußler, Ingo Kleiter, Marius Ringelstein, Orhan Aktas, Tania Kümpfel, Daniel Engels, Joachim Havla, Martin W Hümmert, Julian Reza Kretschmer, Daria Tkachenko, Corinna Trebst, Ana Beatriz Ayroza Galvão Ribeiro Gomes, Anne-Katrin Pröbstel, Mirjam Korporal-Kuhnke, Brigitte Wildemann, Sven Jarius, Refik Pul, Mosche Pompsch, Markus Krämer, Florian Then Bergh, Clemens Gödel, Patricia Schwarz, Markus C Kowarik, Paulus Stefan Rommer, Ioannis Vardakas, Makbule Senel, Alexander Winkelmann, Nele Retzlaff, Martin S Weber, Leila Husseini, Annette Walter, Patrick Schindler, Judith Bellmann-Strobl, Friedemann Paul, Ralf Gold, Ilya Ayzenberg\",\"doi\":\"10.1136/jnnp-2024-334863\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Incomplete attack remission is the main cause of disability in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Apheresis therapies such as plasma exchange and immunoadsorption are widely used in neuroimmunology. Data on apheresis outcomes in MOGAD attacks remain limited.</p><p><strong>Methods: </strong>We retrospectively evaluated all apheresis treated attacks occurring in patients with MOGAD between 2008 and 2023 at 18 Neuromyelitis Optica Study Group centres. Treatment response was categorised as complete, partial or no remission. Preattack and follow-up Expanded Disability Status Scale (EDSS) and visual Functional System Scores (FSS) were used to calculate absolute outcomes (ΔEDSS/Δvisual FSS). Predictors of complete remission were analysed using a generalised linear mixed model.</p><p><strong>Results: </strong>Apheresis was used for 117/571 (20.5%) attacks in 85/209 (40.7%) patients. Attacks with simultaneous optic neuritis and myelitis were treated more often with apheresis (42.4%, n=14) than isolated myelitis (25.2%, n=35), cerebral manifestation (21.0%, n=17) or isolated optic neuritis (17.6%, n=51). Apheresis was initiated as first-line therapy in 12% (4.5 (IQR 0-11) days after attack onset), second-line therapy in 62% (15 (IQR 6.75-31) days) and third-line therapy in 26% (30 (IQR 19-42) days). Complete remission was achieved in 21%, partial remission in 70% and no remission in 9% of patients. First-line apheresis (OR 2.5, p=0.040) and concomitant disease-modifying therapy (OR 1.5, p=0.011) were associated with complete remission. Both parameters were also associated with a favourable ΔEDSS. No differences in outcomes were observed between the apheresis types.</p><p><strong>Conclusion: </strong>Apheresis is frequently used in MOGAD attacks. An early start as first-line therapy and concomitant disease-modifying therapy predict full attack recovery.</p>\",\"PeriodicalId\":16418,\"journal\":{\"name\":\"Journal of Neurology, Neurosurgery, and Psychiatry\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":8.7000,\"publicationDate\":\"2024-11-04\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Neurology, Neurosurgery, and Psychiatry\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1136/jnnp-2024-334863\",\"RegionNum\":1,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Neurology, Neurosurgery, and Psychiatry","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1136/jnnp-2024-334863","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Apheresis therapies in MOGAD: a retrospective study of 117 therapeutic interventions in 571 attacks.
Background: Incomplete attack remission is the main cause of disability in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Apheresis therapies such as plasma exchange and immunoadsorption are widely used in neuroimmunology. Data on apheresis outcomes in MOGAD attacks remain limited.
Methods: We retrospectively evaluated all apheresis treated attacks occurring in patients with MOGAD between 2008 and 2023 at 18 Neuromyelitis Optica Study Group centres. Treatment response was categorised as complete, partial or no remission. Preattack and follow-up Expanded Disability Status Scale (EDSS) and visual Functional System Scores (FSS) were used to calculate absolute outcomes (ΔEDSS/Δvisual FSS). Predictors of complete remission were analysed using a generalised linear mixed model.
Results: Apheresis was used for 117/571 (20.5%) attacks in 85/209 (40.7%) patients. Attacks with simultaneous optic neuritis and myelitis were treated more often with apheresis (42.4%, n=14) than isolated myelitis (25.2%, n=35), cerebral manifestation (21.0%, n=17) or isolated optic neuritis (17.6%, n=51). Apheresis was initiated as first-line therapy in 12% (4.5 (IQR 0-11) days after attack onset), second-line therapy in 62% (15 (IQR 6.75-31) days) and third-line therapy in 26% (30 (IQR 19-42) days). Complete remission was achieved in 21%, partial remission in 70% and no remission in 9% of patients. First-line apheresis (OR 2.5, p=0.040) and concomitant disease-modifying therapy (OR 1.5, p=0.011) were associated with complete remission. Both parameters were also associated with a favourable ΔEDSS. No differences in outcomes were observed between the apheresis types.
Conclusion: Apheresis is frequently used in MOGAD attacks. An early start as first-line therapy and concomitant disease-modifying therapy predict full attack recovery.
期刊介绍:
The Journal of Neurology, Neurosurgery & Psychiatry (JNNP) aspires to publish groundbreaking and cutting-edge research worldwide. Covering the entire spectrum of neurological sciences, the journal focuses on common disorders like stroke, multiple sclerosis, Parkinson’s disease, epilepsy, peripheral neuropathy, subarachnoid haemorrhage, and neuropsychiatry, while also addressing complex challenges such as ALS. With early online publication, regular podcasts, and an extensive archive collection boasting the longest half-life in clinical neuroscience journals, JNNP aims to be a trailblazer in the field.