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Public health situation of CIDP patients in nine German centers—neuritis network Germany
No full textZusammenfassung
Hintergrund
Die Diagnose und Behandlung von Patienten mit immunvermittelten Polyneuropathien ist aufgrund der Heterogenität der Erkrankungen herausfordernd.
Ziel der Arbeit
Ein aktueller epidemiologischer Überblick über die Versorgungssituation von Patienten mit immunvermittelten Polyneuropathien innerhalb des deutschen Neuritis-Netzwerks „Neuritis Netz“.
Material und Methoden
Es erfolgte eine Umfrage in neun deutschen neurologischen Zentren, die auf die Betreuung von Patienten mit Immunneuropathie spezialisiert sind. Wir erfassten Diagnose, Vorgehen in der Diagnostik und Nachsorge, typische Symptome bei Manifestation und im Krankheitsverlauf sowie Therapiedaten.
Ergebnisse
Die Erhebung umfasst Daten von 1529 jährlich behandelten Patienten mit Immunneuropathien, 1320 davon mit chronisch inflammatorisch demyelinisierender Polyneuropathie (CIDP). Die Diagnostik umfasste fast immer Lumbalpunktionen sowie Elektroneuro- und -myografien entsprechend den aktuellen Leitlinien. Der Einsatz von Ultraschall, Biopsie und MRT war unterschiedlich. Wichtigster klinischer Parameter zum Therapiemonitoring in allen Zentren war die motorische Funktion in den klinischen Nachuntersuchungen. Zur Erhaltungstherapie wurde bei rund 15 % der Patienten ein breites Spektrum unterschiedlicher Immunsuppressiva eingesetzt.
Diskussion
Die Studie liefert wichtige epidemiologische Daten zur aktuellen Versorgungsituation von Patienten mit Immunneuropathien in Deutschland. Die Weiterentwicklung spezifischer Empfehlungen zur Therapie und Nachverfolgung von CIDP-Patienten ist notwendig, um einen einheitlichen Standard der Patientenversorgung zu gewährleisten. Dieses wird durch die strukturierte Zusammenarbeit von Exzellenzzentren wie dem deutschen Neuritis Netz erheblich unterstützt.Abstract
Background
Diagnosis and treatment of patients with immune-mediated neuropathies is challenging due to the heterogeneity of the diseases.
Objectives
To assess similarities and differences in the current care of patients with immune-mediated polyneuropathies in specialized centers in Germany within the German neuritis network “Neuritis Netz”.
Material and methods
We conducted a cross-sectional survey of nine neurological departments in Germany that specialize in the care of patients with immune-mediated neuropathies. We assessed the diagnosis, the approach to diagnostic work-up and follow-up, typical symptoms at manifestation and progression of the disease, and treatment data.
Results
This report includes data from 1529 patients per year treated for immune-mediated neuropathies, of whom 1320 suffered from chronic inflammatory demyelinating polyneuropathy (CIDP). Diagnostic work-up almost always included nerve conduction studies, electromyography, and lumbar puncture in accordance with current guidelines. The use of ultrasound, biopsy, and MRI varied. The most important clinical parameter for therapy monitoring in all centers was motor function in the clinical follow-up examinations. A wide range of different immunosuppressants was used for maintenance therapy in about 15% of patients.
Conclusions
These data provide important epidemiological insights into the care of patients with immune-mediated neuropathies in Germany. The further development of specific recommendations for treatment and follow-up examinations is necessary to ensure a uniform standard of patient care. This effort is greatly facilitated by a structured collaboration between expert centers such as Neuritis Netz
Biomarkers for therapeutic stratification of patients with myasthenia gravis
No full textDie Myasthenia gravis (MG) ist die häufigste neuromuskuläre Autoimmunerkrankung, die antikörpervermittelt zu einer Störung der neuromuskulären Übertragung und klinisch zu einer belastungsabhängigen Muskelschwäche führt.
Mit dem Einzug moderner Therapieoptionen eröffnen sich viele neue therapeutische Optionen für die MG. Eine verbesserte Behandlung der MG-Patient*innen ist dringend notwendig, da deren Lebensqualität und Teilhabe am Leben gerade auch im langfristigen Verlauf erheblich eingeschränkt ist. Aktuell Fehlen prädiktive Biomarkern, die nicht nur den Erkrankungsverlauf, sondern auch das individuelle Ansprechen auf die verschiedenen Therapien vorhersagen können.
Im Rahmen dieser Habilitationsschrift sind die Ergebnisse meiner bisherigen Arbeiten zur Analyse von klinischen Risikofaktoren für einen schlechten MG-Verlauf sowie Biomarker-Studien zur Erfassung der Erkrankungsaktivität und möglichen Therapie-Ansprechens zusammenfassend dargelegt, welche das Ziel haben einer individualisierten Patientenversorgung näher zu kommen.
Dabei konnte als klinischer Risikofaktor für einen hochaktiven Erkrankungsverlauf im Sinne der Prädiktion einer myasthenen Krise, die Erkrankungsschwere zum Diagnosezeitpunkt festgestellt werden. Mit Hilfe der Daten des Deutschen Myasthenie- Registers konnten wir zu Beginn der COVID-19 Pandemie zeigen, dass MG-Patient*innen mit einer COVID-19-Infektion und bestehender immunsuppressiver Therapie ein schlechteres Outcome gemessen an der Rate der Hospitalisation und insbesondere eine höhere, krankheitsspezifische Letalität im Vergleich zu anderen Autoimmunerkrankungen aufweisen.
In Bezug auf mögliche Biomarker konnten drei Kandidaten detektiert werden: Serum Calprotectin ist ein etablierter Marker der mikrobiellen Dysbiose und korreliert mit der klinisch aktiven MG. Dieser Marker könnte daher helfen neben den klinischen Skalen, die Krankheitsaktivität zu bestimmen. Darüber hinaus unterstreicht diese Studie die pathophysiologische Bedeutung der Darm-Dysbiose für die MG und bietet die Grundlage zur weiteren Erforschung möglicher neuer modulierender Therapien des Darmmikrobioms. Serum Neurofilament light chain ist insbesondere bei der Acetylcholinrezeptor-(AChR)-Ak positiven MG deutlich erhöht und könnte als prognostischer Marker, v.a. bei hochaktiven Verläufen unter intensivierter Therapie eingesetzt werden, um eine irreversible Destruktion an der neuromuskulären Endplatte zu vermeiden. Komplementaktivierungsmarker sind bei der AChR-Ak positiven MG erhöht und sinken unter immunsuppressiver Therapie. Die Messung der Komplementaktivierung könnte daher ein möglicher prädiktiver Biomarker zur Detektion des therapeutischen Ansprechens für die AChR-Ak positiven MG darstellen.Myasthenia gravis (MG) is the most common neuromuscular autoimmune disease that leads to an antibody-mediated disruption of neuromuscular transmission and clinically to exercise-induced muscle weakness.
The advent of modern treatment options has opened many new therapeutic options for MG. Improved treatment of MG patients is urgently needed, as their quality of life and participation is considerably restricted. Currently, there is a lack of predictive biomarkers that can detect disease activity and response to therapies.
This habilitation thesis summarizes the results of my previous work on the analysis of clinical risk factors for highly active MG disease course as well as biomarker studies to detect disease activity and therapy response, with the aim of coming closer to individualized patient care.
The severity of the disease at the time of diagnosis was identified as the strongest predictive clinical risk factor for developing a myasthenic crisis. Using data from the German Myasthenia Registry, we were able to show at the beginning of the COVID-19 pandemic that MG patients with a COVID-19 infection and existing immunosuppressive therapy have a poorer outcome measured by the rate of hospitalization and, in particular, a higher disease-specific mortality rate compared to other autoimmune diseases.
With regard to possible biomarkers, three potential candidates were identified: serum calprotectin is an established marker of microbial dysbiosis and correlates with clinically active MG. This marker could therefore help to determine disease activity in addition to the common clinical scales. Furthermore, this study underlines the pathophysiological significance of gut dysbiosis for MG and provides the basis for further research into potential new modulating therapies of the gut microbiome. Serum neurofilament light chain is particularly elevated in acetylcholine receptor (AChR)-Ak positive MG and could be used as a prognostic marker, especially in highly active MG patients under intensified therapy, to avoid irreversible destruction of the neuromuscular endplate. Complement activation markers are elevated in AChR-Ak positive MG and decrease under immunosuppressive therapy. The measurement of complement activation could therefore be a possible predictive biomarker for detecting treatment response in AChR-Ak positive MG
C5 complement inhibition versus FcRn modulation in generalised myasthenia gravis
No full textBackground Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular junctions, leading to fluctuating muscle weakness. While many patients respond well to standard immunosuppression, a substantial subgroup faces ongoing disease activity. Emerging treatments such as complement factor C5 inhibition (C5IT) and neonatal Fc receptor (FcRn) antagonism hold promise for these patients. However, the current landscape is hindered by a paucity of comparative data that is crucial for treatment decisions. Objective This study aims to compare the effectiveness and safety of C5IT and FcRn antagonists in a real-world setting. Methods A retrospective analysis of 153 MG patients from 8 German specialised MG centres receiving either C5IT (26 eculizumab, 80 ravulizumab) or efgartigimod (47 patients) was conducted. Propensity score matching (PSM) was employed to compare changes in MG-specific outcome parameters within the first 6 months after treatment initiation, along with safety profiles and concomitant MG therapy. Results Both treatment strategies led to rapid clinical improvements and substantial reductions in prednisolone doses. However, insufficient response was noted in 20%–49.1% of patients based on Quantitative MG and MG Activities of Daily Living (MG-ADL) scores. We did not identify any new safety concerns. After PSM, 40 patients remained in each group. In both cohorts, reductions in MG-ADL as prespecified primary study endpoint were comparable. Moreover, analyses of secondary outcome parameters demonstrated similar results for C5IT versus FcRn. Conclusion In contrast to current meta-analyses and indirect comparisons of clinical trial data, our real-world study demonstrates comparable efficacy and safety of C5IT and FcRn antagonism in MG
Going Beyond Counting First Authors in Author Co-citation Analysis
Get PDFThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Get PDF“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Get PDFWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Dispelling the Myths Behind First-author Citation Counts
Get PDFWe conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
koamabayili/VECTRON-author-checklist: VECTRON author checklist
No full textWe have done our best to complete the author checklist relating to the use of animals in the hut study. Note that the objective for the hut study was to evaluate the IRS treatment applications for residual efficacy against Anopheles mosquitoes, including the local An. coluzzii mosquito population. Cows were only used to attract mosquitoes into the huts and no tests were carried out directly on the cows. The author checklist is intended for use with studies where experiments are carried out on animals, which is why we have had such difficulty in completing this for the hut study, as many of the questions do not relate to how the cows were used
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