ABSTRACT
Objective To explore the mechanisms underlying the mediating effect of regulatory B (Breg)cells in the pathogenesis of pemphigus.Methods Peripheral blood specimens were collected from 48 patients with pemphigus at different degrees of severity and 20 healthy controls.Flow cytometry was conducted to analyze the frequency of Breg(CD19 +CD24hiCD38hi)cells in peripheral CD19+ B cells,enzyme linked immunosorbent assay(ELISA)to determine the titer and subtypes of anti-desmoglein(Dsg)1 and 3 antibodies in sera from these subjects.The relationship of anti-Dsg 1 and 3 antibodies with the frequency of Breg cells was assessed by Spearman's rank correlation test.Results Significant differences were observed in the proportion of Breg cells in CD19+ B cells between patients with pemphigus and healthy controls(11.54％ ± 0.97％ vs.8.19％ ± 0.85％,P =0.04),as well as between patients with acute mild pemphigus and those with moderate and severe pemphigus(5.17％ ± 2.14％ vs.11.38％ ± 5.30％ and 11.17％ ± 5.31％,P =0.042,0.046,respectively).The frequency of Breg cells was positively correlated with the absorbance value for anti-Dsg1 antibodies of IgG4 subclass and IgG4/IgG1 ratio(r =0.527,0.565,respectively,both P ＜ 0.01).Conclusions Breg cells are mainly associated with the production of antibodies in and disease severity of pemphigus,while the actual mechanism of action remains unknown.
ABSTRACT
Objective To assess the correlation between the subclasses of antibodies against desmoglein (Dsg) 1 and Dsg3 and disease activity in patients with pemphigus. Methods Sera were collected from 47 patients with pemphigus, and ELISA was performed to determine the titers and subclasses of antibodies against Dsg1 and Dsg3. The correlation of antibody titers and subclasses with disease activity was assessed.Results Clinical phenotype was associated with antibody profiles in these patients with pemphigus. Of 17 patients with mucocutaneous involvement, 14 (82.4%) had both anti-Dsg3 and anti-Dsg1 antibodies; of 16 patients with cutaneous involvement, 15 (93.7%) had anti-Dsg1 antibody, only 1 (6.3%) developed anti-Dsg3 antibody; of 6 patients with mucosal involvement, all ( 100% ) had only anti-Dsg3 antibody. The serum levels of antibodies against Dsg1 and Dsg3 were increased, but not in parallel with the disease severity in these patients.Moreover, the subclasses of antibodies were correlated with disease severity. IgG4 subclass antibodies against Dsg1 and Dsg3 predominated in patients with pemphigus at active stage, whereas IgG1 subclass in those at remission stage. The serum levels (expressed as absorbance value) of IgG4 and IgG1 subclass antibodies were 1.92 ± 1.21 and 0.60 ± 0.61 respectively, with the ratio of IgG4 to IgG1 more than 1, in patients with antiDsg1 antibodies at active stage, 0.03 ± 0.02 and 0.22 ± 0.11 respectively with the ratio of IgG4 to IgG1 less than 1, in those at remission stage, 2.35 ± 2.17 and 1.84 ± 1.16 respectively with the ratio of IgG4 to IgG1 more than 1 in patients with anti-Dsg3 antibodies at active stage, 0.15 ± 0.16 and 1.05 ± 0.77 respectively with the ratio of IgG4 to IgG1 less than 1 in those at remission stage. Conclusions The subclasses of pemphigus-specific antibodies are closely correlated with the disease severity in patients with pemphigus. The detection of subclasses and titers of anti-Dsg1 and anti-Dsg3 antibodies may aid in the diagnosis of and monitoring of disease severity in pemphigus.