B Cell Profiling in Patients with Pemphigus Vulgaris

Abstract

Pemphigus vulgaris is a severe, socially significant autoimmune disease associated with autoantibodies to the desmoglein 3 antigen. The disease affects all age groups, beginning at 18 years of age; the mortality rate of pemphigus can reach as high as 50%, depending on a patients age and a number of other factors. There is no highly selective or personalized therapy for pemphigus vulgaris at the moment. One of the well-known therapeutic approaches to the disease is to use rituximab, an anti-CD20 antibody that can help achieve B cell depletion in peripheral blood. To solve the problem of nonspecific elimination of B cells in patients with pemphigus vulgaris, it is reasonable to use specific immunoligands, their choice being based on an assessment of the level of autoantibodies specific to each of the fragments of desmoglein. In this work, the proportion of autoreactive B cells in patients diagnosed with pemphigus vulgaris is found to be 0.090.16%; a positive correlation was revealed between the antibody level and the number of autoreactive B cells to various fragments of desmoglein.

From neglect to spotlight: the underappreciated role of B cells in cutaneous inflammatory diseases

“…Remarkably, the disease severity of PV has been correlated with the abundance of serum DSG1/3-specific autoantibody. The well-characterized antigenic specificity for DSG1/3 has allowed researchers to isolate these pathogenic B cells from the peripheral blood of PV patients, thus elucidating their distinctive transcriptomic and phenotypic profiles ( 51 – 53 ). Detection of DSG1/3-specific B cells in PV patients demonstrated that DSG1/3-specific memory B cells also correlate with disease severity ( 51 , 52 ).…”