Abstract
Background: B-cell depletion therapy was demonstrated to be a valid and safe alternative as an adjuvant in oral-pharyngeal pemphigus vulgaris (OPV) patients. We aimed to assess its effects on anti-desmoglein (Dsg) 1 and 3 and leukocytes subsets profile in these patients’ population. Methods and Materials: We evaluated the immunologic profile of 10 OPV patients treated with RTX as adjuvant by using the ELISA testing for anti-Dsg-1 and -3 titers and the immunophenotyping for B and T-cell lymphocyte subpopulations and compared them with the PDAI score for clinical remission. Results: A significant difference in medians between baseline, end of RTX therapy, and 6 months after RTX therapy was observed in Dsg-3 titer (p < 0.001), in the CD8 (p = 0.009), and CD20 counts (p < 0.001). Multiple comparisons after Bonferroni adjustment confirmed such significant differences mainly between baseline and the end of RTX therapy and baseline and 6 months after RTX therapy. Only the anti-Dsg-3 titer at the end of RTX therapy demonstrated a slight positive correlation with the PDAI score at baseline (p = 0.046, r = 0.652). Conclusions: B-cell depletion adjuvant therapy in OPV patients demonstrated a significant impact on anti-Dsg-3 titer and B and T-cell lymphocyte subpopulations profile.
Highlights
Pemphigus Vulgaris (PV) is a potentially fatal autoimmune mucocutaneous blistering disease characterized by a suprabasal intraepithelial detachment [1]
First-line treatment of PV has always been based on conventional immunosuppressive therapy (CIST) with a high dose of corticosteroids (CS) and CS-sparing agents, such as azathioprine, cyclophosphamide, or mycophenolic acid, in order to reduce the total dose of CS and minimize mortality and morbidity [4,5]
The use of RTX in the treatment of PV has been rapidly increasing over the past years, yielding very important results in the attainment of long-lasting remission in the majority of patients that could no longer be treated with CIST [22]
Summary
Pemphigus Vulgaris (PV) is a potentially fatal autoimmune mucocutaneous blistering disease characterized by a suprabasal intraepithelial detachment [1]. This phenomenon, known as acantholysis, is caused by a humoral response against a broad spectrum of antigens responsible for cell-cell adhesions [1]. First-line treatment of PV has always been based on conventional immunosuppressive therapy (CIST) with a high dose of corticosteroids (CS) and CS-sparing agents, such as azathioprine, cyclophosphamide, or mycophenolic acid, in order to reduce the total dose of CS and minimize mortality and morbidity [4,5]. Prolonged use of Biomolecules 2021, 11, 1634. The introduction of biologic agents, such as intravenous immunoglobulins G (IVIgG) Biomolecules 2021, 11, 1634 these medications has been associated with significant unwanted side effects [5] and/or sometimes a failure in achieving a complete clinical and/or immunological remission [6].
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