TY - JOUR
T1 - The pathogenic activity of anti-desmoglein autoantibodies parallels disease severity in rituximab-treated patients with pemphigus vulgaris
AU - Sinistro, Anna
AU - Calabresi, Valentina
AU - Lupi, Francesca
AU - Sera, Francesco
AU - Frezzolini, Alessandra
AU - Ruffelli, Marina
AU - De Pità, Ornella
AU - Camaioni, Diana
AU - Cianchini, Giuseppe
AU - Di Zenzo, Giovanni
PY - 2015/11/1
Y1 - 2015/11/1
N2 - Background: Pemphigus vulgaris (PV) is an autoimmune blistering disease mediated by IgG autoantibodies targeting desmogleins (Dsgs). The anti-CD20 monoclonal antibody rituximab is increasingly used in corticosteroid-resistant PV patients. In a subset of rituximab-treated patients in remission, high ELISA index values have been reported; however, their significance remains so far unclear. Objective: To address the discrepancy between anti-Dsg3 serum antibody titers and disease severity. Materials & methods: 6 rituximab-treated PV patients were prospectively followed-up for two years and anti-Dsg3 autoantibodies levels and pathogenic activity were measured. Results: All patients achieved complete remission without any serious side effects. Both anti-Dsg3 autoantibodies (p = 0.031) and their pathogenic activity (p = 0.003) were significantly related to disease severity. However, in selected patients, the dissociation index was a more sensitive indicator for PV clinical activity than the ELISA index. Conclusion: Our findings have demonstrated the existence of non-pathogenic autoantibodies in PV patients in remission, establishing the basis for the design of a system able to precisely monitor the course of disease.
AB - Background: Pemphigus vulgaris (PV) is an autoimmune blistering disease mediated by IgG autoantibodies targeting desmogleins (Dsgs). The anti-CD20 monoclonal antibody rituximab is increasingly used in corticosteroid-resistant PV patients. In a subset of rituximab-treated patients in remission, high ELISA index values have been reported; however, their significance remains so far unclear. Objective: To address the discrepancy between anti-Dsg3 serum antibody titers and disease severity. Materials & methods: 6 rituximab-treated PV patients were prospectively followed-up for two years and anti-Dsg3 autoantibodies levels and pathogenic activity were measured. Results: All patients achieved complete remission without any serious side effects. Both anti-Dsg3 autoantibodies (p = 0.031) and their pathogenic activity (p = 0.003) were significantly related to disease severity. However, in selected patients, the dissociation index was a more sensitive indicator for PV clinical activity than the ELISA index. Conclusion: Our findings have demonstrated the existence of non-pathogenic autoantibodies in PV patients in remission, establishing the basis for the design of a system able to precisely monitor the course of disease.
KW - Autoantibodies
KW - Desmoglein 3
KW - Disease severity
KW - ELISA
KW - Pathogenicity
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U2 - 10.1684/ejd.2015.2652
DO - 10.1684/ejd.2015.2652
M3 - Article
AN - SCOPUS:84957869319
VL - 25
SP - 578
EP - 585
JO - European Journal of Dermatology
JF - European Journal of Dermatology
SN - 1167-1122
IS - 6
ER -