Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful Diagnosis
Neuromuscular junction disorders (NJDs) are a heterogeneous group of diseases including myasthenia gravis (MG). In some cases, patients are present with myasthenic symptoms without evidence of autoimmune antibodies, making diagnosis challenging. Total plasma exchange (TPE) has proven efficacy in NJD...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-07-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/11/15/4383 |
| _version_ | 1797413452878184448 |
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| author | Andreas Totzeck Michael Jahn Benjamin Stolte Andreas Thimm Christoph Kleinschnitz Tim Hagenacker |
| author_facet | Andreas Totzeck Michael Jahn Benjamin Stolte Andreas Thimm Christoph Kleinschnitz Tim Hagenacker |
| author_sort | Andreas Totzeck |
| collection | DOAJ |
| description | Neuromuscular junction disorders (NJDs) are a heterogeneous group of diseases including myasthenia gravis (MG). In some cases, patients are present with myasthenic symptoms without evidence of autoimmune antibodies, making diagnosis challenging. Total plasma exchange (TPE) has proven efficacy in NJDs. The objective is to describe the safety and efficacy of TPE in NJD patients with questionable disease activity or uncertain diagnosis in order to assess the diagnostic potential of TPE. We report an observational, retrospective cohort study of clinical routine data. All the data were derived from the electronic medical records of the Department of Neurology at University Hospital Essen. We searched for patients with NJDs between 1 July 2018 and 30 June 2021. Of the 303 patients who presented to the department with NJDs, 20 were treated with TPE; 9 patients did not show a measurable benefit from TPE (45%), 6 of whom were diagnosed with seronegative MG. Of these, 3 (50%) had long-standing ocular symptoms. There were decreases in the mean arterial pressure, hemoglobin, hematocrit and fibrinogen during treatment, which were not considered clinically relevant. In (seronegative) myasthenic patients, TPE may help to verify an uncertain diagnosis or to reveal possible muscle damage, allowing unnecessary therapy to be avoided. |
| first_indexed | 2024-03-09T05:19:09Z |
| format | Article |
| id | doaj.art-6a140a8ad3774e5783ff1098ff77eacf |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-09T05:19:09Z |
| publishDate | 2022-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-6a140a8ad3774e5783ff1098ff77eacf2023-12-03T12:42:53ZengMDPI AGJournal of Clinical Medicine2077-03832022-07-011115438310.3390/jcm11154383Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful DiagnosisAndreas Totzeck0Michael Jahn1Benjamin Stolte2Andreas Thimm3Christoph Kleinschnitz4Tim Hagenacker5Department of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, 45147 Essen, GermanyDepartment of Nephrology, University Hospital Essen, University of Duisburg-Essen, 45147 Essen, GermanyDepartment of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, 45147 Essen, GermanyDepartment of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, 45147 Essen, GermanyDepartment of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, 45147 Essen, GermanyDepartment of Neurology and Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University Hospital Essen, University of Duisburg-Essen, 45147 Essen, GermanyNeuromuscular junction disorders (NJDs) are a heterogeneous group of diseases including myasthenia gravis (MG). In some cases, patients are present with myasthenic symptoms without evidence of autoimmune antibodies, making diagnosis challenging. Total plasma exchange (TPE) has proven efficacy in NJDs. The objective is to describe the safety and efficacy of TPE in NJD patients with questionable disease activity or uncertain diagnosis in order to assess the diagnostic potential of TPE. We report an observational, retrospective cohort study of clinical routine data. All the data were derived from the electronic medical records of the Department of Neurology at University Hospital Essen. We searched for patients with NJDs between 1 July 2018 and 30 June 2021. Of the 303 patients who presented to the department with NJDs, 20 were treated with TPE; 9 patients did not show a measurable benefit from TPE (45%), 6 of whom were diagnosed with seronegative MG. Of these, 3 (50%) had long-standing ocular symptoms. There were decreases in the mean arterial pressure, hemoglobin, hematocrit and fibrinogen during treatment, which were not considered clinically relevant. In (seronegative) myasthenic patients, TPE may help to verify an uncertain diagnosis or to reveal possible muscle damage, allowing unnecessary therapy to be avoided.https://www.mdpi.com/2077-0383/11/15/4383myasthenia gravisLambert–Eaton syndromedecrementMGFAQMG |
| spellingShingle | Andreas Totzeck Michael Jahn Benjamin Stolte Andreas Thimm Christoph Kleinschnitz Tim Hagenacker Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful Diagnosis Journal of Clinical Medicine myasthenia gravis Lambert–Eaton syndrome decrement MGFA QMG |
| title | Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful Diagnosis |
| title_full | Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful Diagnosis |
| title_fullStr | Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful Diagnosis |
| title_full_unstemmed | Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful Diagnosis |
| title_short | Total Plasma Exchange in Neuromuscular Junction Disorders—A Single-Center, Retrospective Analysis of the Efficacy, Safety and Potential Diagnostic Properties in Doubtful Diagnosis |
| title_sort | total plasma exchange in neuromuscular junction disorders a single center retrospective analysis of the efficacy safety and potential diagnostic properties in doubtful diagnosis |
| topic | myasthenia gravis Lambert–Eaton syndrome decrement MGFA QMG |
| url | https://www.mdpi.com/2077-0383/11/15/4383 |
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