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

University Hospital, Strasbourg, France

Status

Not yet enrolling

Conditions

ANCA-associated Vasculitis

Antiphospholipid Syndrome

Primary Immunodeficiencies

Systemic Scleroderma

Systemic Lupus Erythematosus

Treatments

Biological: blood draw

Study type

Observational

Funder types

Other

Identifiers

NCT07251179

9942 (Other Identifier)

Details and patient eligibility

About

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.

Enrollment

200 estimated patients

Sex

All

Ages

18 to 70 years old

Volunteers

No Healthy Volunteers

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