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Updated: Jun 11, 2025

Isolating Human Peripheral Blood Mononuclear Cells and CD4+ T cells from Sézary Syndrome Patients for Transcriptomic Profiling

Isolating Human Peripheral Blood Mononuclear Cells and CD4+ T cells from Sézary Syndrome Patients for Transcriptomic Profiling

Published on: October 14, 2021

[Update on Behçet syndrome].

Ina Kötter^1,2, Nikolas Ruffer³, Martin Krusche³

¹III. Medizin, Sektion für Rheumatologie und Entzündliche Systemerkrankungen, Universitätsklinikum Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Deutschland. i.koetter@uke.de.

Zeitschrift Fur Rheumatologie

|October 1, 2024

Summary

This summary is machine-generated.

Behçet syndrome (BS) is now classified as an MHC-I-opathy, bridging autoinflammatory and autoimmune conditions. Recent advancements include refined diagnostic criteria and novel therapeutic targets for this complex vasculitis.

Keywords:

Diagnosis Differential diagnosis Pathophysiology Treatment Vasculitis

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Area of Science:

Immunology
Rheumatology
Genetics

Context:

Behçet syndrome (BS) is a multisystem vasculitis with complex pathophysiology.
Understanding BS has evolved, positioning it between autoinflammatory and autoimmune diseases.
Recent research focuses on genetic associations and clinical manifestations.

Purpose:

To provide an overview of recent innovations in Behçet syndrome research and clinical practice.
To highlight advancements in understanding BS pathophysiology and diagnosis.
To summarize new therapeutic approaches and diagnostic tools.

Summary:

Behçet syndrome is now classified as an MHC-I-opathy, emphasizing its strong association with HLA class I antigens and a role in pathogenesis.
Established diagnostic criteria (ICBD 2014) are effective but may require adjustments in low-prevalence regions.
New insights reveal disease clusters varying by country and age, alongside improved diagnostic methods like specific venous sonography and enhanced pathergy testing.
Treatment advances include successful use of tocilizumab, secukinumab, JAK inhibitors, and ustekinumab in refractory cases, with updated EULAR recommendations anticipated.

Impact:

Clarifies the pathophysiological classification of Behçet syndrome, aiding in targeted research.
Offers updated diagnostic strategies and tools for improved accuracy in BS identification.
Introduces novel therapeutic agents and strategies for managing treatment-refractory Behçet syndrome.
Provides a foundation for future research and clinical guideline development in Behçet syndrome management.