Characterization of Autoreactive b Lymphocytes in Autoimmune Diseases and Immune Deficiencies (AutoB-Tetramer)

Autoimmune diseases (AID), whether systemic or organ-specific, affect approximately one in ten people, and their prevalence continues to increase. Many AIDs are linked to the emergence of autoreactive B cells (BCs) directed against components of the self. In healthy individuals, these autoreactive B cells are counter-selected or regulated before reaching the antibody-secreting cell compartment. However, in predisposed individuals, a breakdown in B cell tolerance can occur, leading to the formation of autoantibodies with devastating consequences, such as the emergence of systemic lupus erythematosus (SLE), rheumatoid arthritis, and vasculitis. B-cell depletion is often beneficial in these patients, but paradoxically, therapies targeting B cells are not always effective. Furthermore, B cell depletion via LB-specific antibodies (anti-CD20) or treatment with CD19 chimeric antigen receptor T cells (CAR T) leads to complete and prolonged depression of the entire B compartment without targeting the LB population responsible for the onset of the disease. To date, two pitfalls in studies of human autoreactive LBs often complicate the interpretation of results:

i) the difficulty of identifying autoreactive LBs among all LBs, ii) demonstrating the pathogenicity of autoreactive B lymphocytes when they can be identified individually. We propose to quantify and phenotype these autoreactive/pathogenic B cells using high-throughput flow cytometry in several clinical situations.

Study Overview

• Status: Not yet recruiting
• Conditions: Systemic Lupus Erythematosus; Systemic Scleroderma; ANCA-associated Vasculitis; Antiphospholipid Syndrome; Primary Immunodeficiencies
• Intervention / Treatment: Biological: blood draw

• Study Type: Observational
• Enrollment (Estimated): 200

Contacts and Locations

• Study Contact: Name: Anne-Sophie KORGANOW, MD; Phone Number: 0033 03 69 55 09 94; Email: anne-sophie.korganow@chru-strasbourg.fr

Study Locations

• France
  • Strasbourg, France, 67000
    • Hôpitaux Universitaires de Strasbourg
    • Contact: Anne-Sophie KORGANOW, MD; Phone Number: 0033 +33369551123; Email: anne-sophie.korganow@chru-strasbourg.fr
    • Contact: Email: anne-sophie.korganow@chru-strasbourg.fr
    • Principal Investigator: Anne-Sophie KORGANOW, MD

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Hôpitaux Universitaires de Strasbourg

Description

Inclusion Criteria:

• Patients aged between 18 and 70
• Patients for whom at least one of the following conditions has been confirmed:
• Systemic lupus erythematosus meeting the 2019 ACR/EULAR classification criteria.
• Systemic scleroderma meeting the 2013 ACR/EULAR classification criteria. ANCA-associated vasculitis according to the 2022 EULAR/ACR classification criteria.
• Antiphospholipid syndrome according to the 2023 ACR/EULAR criteria.
• Primary immunodeficiencies according to IUIS criteria.
• Patients capable of understanding the objectives of the research.
• Patients affiliated with a social security health insurance scheme (beneficiary or dependant).
• Patients who have signed and dated the informed consent form for non-identifying genetic testing.

Exclusion Criteria:

• Patient refusing to participate in the study
• Patient in a period of exclusion (determined by a previous or ongoing study) Inability to provide the subject with informed consent (in an emergency or immediate life-threatening situation, difficulties in understanding the subject, etc.)
• Patient under legal protection
• Patient under guardianship or conservatorship

Study Plan

How is the study designed?

Number of groups / cohorts

5

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Systemic lupus erythematosus	Biological: blood draw; blood draw
Systemic scleroderma	Biological: blood draw; blood draw
ANCA-associated vasculitis	Biological: blood draw; blood draw
Antiphospholipid syndrome	Biological: blood draw; blood draw
Primary immunodeficiencies	Biological: blood draw; blood draw

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Study of the percentage of autoreactive LB / tetrameric LB+	inclusion visit

Collaborators and Investigators

• Sponsor: University Hospital, Strasbourg, France

Study record dates

Study Major Dates

• Study Start (Estimated): January 1, 2026
• Primary Completion (Estimated): December 31, 2031
• Study Completion (Estimated): December 31, 2031

Study Registration Dates

• First Submitted: November 18, 2025
• First Submitted That Met QC Criteria: November 18, 2025
• First Posted (Estimated): November 26, 2025

Study Record Updates

• Last Update Posted (Estimated): December 4, 2025
• Last Update Submitted That Met QC Criteria: November 26, 2025
• Last Verified: November 1, 2025

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Vascular Diseases; Cardiovascular Diseases; Connective Tissue Diseases; Autoimmune Diseases; Immune System Diseases; Skin Diseases; Skin Diseases, Vascular; Vasculitis; Systemic Vasculitis; Skin and Connective Tissue Diseases; Lupus Erythematosus, Systemic; Scleroderma, Systemic; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Antiphospholipid Syndrome; Investigative Techniques; Specimen Handling; Clinical Laboratory Techniques; Diagnostic Techniques and Procedures; Diagnosis; Punctures; Surgical Procedures, Operative; Blood Specimen Collection
• Other Study ID Numbers: 9942 (CTEP)

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No