Orphanet Journal of Rare Diseases | A 20-Year Bibliometric Analysis of Autoimmune Nodopathy Research

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This article systematically analyzes research trends in Autoimmune Nodopathy (AN) from 2005 to 2025, revealing contributions from key countries, authors, and institutions, and emphasizing the importance of international collaboration and antibody subtyping in disease diagnosis and treatment.

 

Literature Overview
The article titled Global research landscape of autoimmune nodopathy: a 20-year bibliometric analysis (2005–2025), published in the Orphanet Journal of Rare Diseases, reviews and summarizes original research articles on autoimmune nodopathy (AN) from 2005 to 2025. It analyzes research trends, high-impact publications, country and institutional collaboration networks, and uses VOSviewer and CiteSpace for citation burst detection and keyword clustering. Based on literature data, it predicts research output trends for 2030 and 2032. The article summarizes bibliometric characteristics, providing a roadmap for future research and collaboration frameworks.

Background Knowledge
Autoimmune nodopathy (AN) is a rare immune-mediated peripheral neuropathy caused by autoantibodies targeting nodal and paranodal proteins, such as neurofascin-155 and contactin-1. It was formally recognized as a distinct disease by the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) in 2021, previously being classified as a variant of chronic inflammatory demyelinating polyneuropathy (CIDP). Due to the lack of macrophage-mediated demyelination, AN shows poor clinical response to IVIG and corticosteroid treatment and is associated with higher mortality, especially in patients with IgG1 pan-neurofascin antibodies. Current treatment options include immunoglobulin, rituximab, and plasmapheresis, though no standardized treatment exists. Bibliometric analysis shows significant research output from France, Germany, Spain, and Japan, with a notable increase after 2021. Despite rising interest, large-scale epidemiological studies and randomized controlled trials remain lacking, highlighting the need for international collaboration and prospective study designs.

 

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Research Methods and Findings
Researchers conducted a literature search using the Web of Science Core Collection database with search strategies incorporating keywords such as ‘CIDP’, ‘autoimmune nodopathy’, and ‘contactin-1’. A total of 109 original English-language research articles were selected. VOSviewer and CiteSpace were used to analyze collaboration networks and citation bursts, while SPSS and Excel were used for trend prediction. The study also evaluated output and citation data for countries, institutions, authors, and journals, and performed clustering analysis on keywords, highly cited papers, and research types.

Key Conclusions and Insights

• After the EAN/PNS formally defined AN in 2021, the number of publications rapidly increased, reaching 16 in 2022.
• France, Germany, Spain, and Japan are the main contributors, accounting for 66% of total publications.
• High-impact authors include Querol Luis, Sommer Claudia, Doppler Kathrin, primarily affiliated with institutions such as Universitat Autònoma de Barcelona, University of Würzburg, and Aix-Marseille University.
• Highly cited papers focus on antibody profiles, clinical phenotypes, treatment responses, and pathogenic mechanisms. Notably, Querol et al. (2014) on Neurofascin IgG4 antibodies and poor IVIG response received the most citations (265).
• Keyword clustering shows a shift in research focus from general neuropathies like CIDP and Guillain-Barre syndrome to specific molecules such as AN, nodal/paranodal proteins, and IgG4.
• While clinical studies dominate, experimental and neuropathological studies remain valuable for understanding disease mechanisms. However, large-scale randomized controlled trials are still lacking.
• International collaboration networks indicate strong cooperation among European countries, with increasing research output from Asia, suggesting a widening geographical distribution of research efforts.

Significance and Future Directions
This study is the first bibliometric analysis on autoimmune nodopathy, systematically mapping research hotspots, collaboration networks, and citation trends. It highlights the need for multicenter epidemiological studies, prospective treatment trials, correlation analysis between antibody subtypes and treatment responses, and standardized laboratory testing protocols. Establishing international collaboration and patient registries will accelerate mechanistic and clinical translation of AN research.

 

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Conclusion
The article Global research landscape of autoimmune nodopathy: a 20-year bibliometric analysis (2005–2025) is the first systematic bibliometric analysis of autoimmune nodopathy (AN) research. It reveals a rapid increase in publications since the formal recognition of AN by EAN/PNS in 2021, peaking in 2022. Key contributing countries include France, Germany, Spain, and Japan, with high-impact authors from institutions such as Universitat Autònoma de Barcelona and University of Würzburg. Keyword analysis shows a research focus shifting from broad neuropathies like CIDP to molecular features such as AN, nodal proteins, and IgG4. Despite the surge in research, large-scale epidemiological and randomized controlled trials remain absent, necessitating stronger international collaboration, multicenter studies, and patient registries to advance mechanistic and therapeutic understanding. Additionally, the study emphasizes the importance of prospective designs, antibody subtype-treatment correlation, and standardized biosample handling. The bibliometric analysis provides a clear research landscape and serves as a reference for future topic selection and collaboration network construction.

 

Mehmet Ertan Temir and Gizem Yavaş Temir. Global research landscape of autoimmune nodopathy: a 20-year bibliometric analysis (2005–2025). Orphanet Journal of Rare Diseases.