John Soltys¹

¹University of South Alabama

Autoimmune encephalitis remains a diagnostic challenge, yet is appreciated as a “not-to-miss” inflammatory disorder. LGI1-Antibody encephalitis is one of the most common forms of autoimmune encephalitis, and shares many clinical features with CASPR2-Antibody encephalitis. This talk explores the clinical presentation of these disorders, contrasts these presentations to other forms of autoimmune encephalitis, and reviews emerging treatment paradigms based on contemporary advances in the underlying molecular immunopathogenesis.

Adrian Lee^1
1ICPMR Westmead Hospital

Introduction: Sjögren’s disease (SjD) is an autoimmune disease that causes widespread pain, fatigue and dryness symptoms of the eyes and mouth. Pathologically, it is characterised by B cell hyperreactivity, self-reactivity and autoantibodies to nuclear autoantigens. The specific B cell developmental state where self-reactivity arises, is not clear and identifying circulating self-reactive B cells would inform targeted therapy. Hence, we examined five populations within the peripheral blood B cell compartment of patients with SjD for reactivity to nuclear/cellular antigens.

 

Methods: We performed flow cytometry to compare B cells across SjD patients and healthy donors. Five blood B cell populations: transitional, mature naïve, switched memory, double negative and plasmablasts from 5 SjD patients were single cell-sorted and cultured for two weeks. Supernatants were assessed for self-reactivity using ELISA and flow cytometry of permeabilised HEK293 cells. 

 

Results: Early (antigen-inexperienced) B cells were expanded in the blood of SjD patients and displayed evidence for high self-reactivity compared to corresponding populations in healthy donors. 

 

Conclusions: Consistent with their autoimmune diathesis, a greater degree of nuclear/cellular self-reactivity was seen in the circulating B cell populations of SjD patients.