018 Evolution of autoreactive B and T cells in pemphigus patients with Rituximab or corticosteroida regimen treatment

Pemphigus is an autoimmune blistering disease mediated by autoantibodies (Abs) directed against desmogleins (Dsg). We recently showed that first line treatment with Rituximab (RTX) was more effective than standard oral corticosteroid (CS). To understand the immunological mechanisms that mediate the long-lasting clinical remission (CR) after RTX treatment, we analyzed the phenotype and antigen specificity of B cells and T follicular helper cells (TFH) by flow cytometry and the number of Dsg+ IgG Abs Secreting Cell (ASC) by ELISPOT.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Adaptive Immunity and Autoimmunity Source Type: research

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Authors: Pires CAA, Einecke YS, Pereira NG, Costa CCC, Cunha ACDS, Moreira AG, Rodrigues FDN, Xavier MB Abstract Background: Pemphigus are rare chronic autoimmune blistering disorders with challenging therapeutic management.Objective: To investigate the therapeutic outcomes of pemphigus patients admitted to a dermatology referral service in northern Brazil.Materials and methods: We conducted a retrospective analysis of 32 patients with histopathological diagnosis of pemphigus between 2010 and 2016. Clinical and epidemiological data were collected. Therapeutic outcome was evaluated according to the criteria proposed...
Source: Journal of Dermatological Treatment - Category: Dermatology Tags: J Dermatolog Treat Source Type: research
ConclusionWhile corticosteroid in combination with either AZA or MMF is an effective therapeutic regimen for the treatment of pemphigus, MMF demonstrates a shorter time to achieve CR on therapy and has a significantly higher steroid-sparing effect.
Source: Dermatology and Therapy - Category: Dermatology Source Type: research
ConclusionsAdjunctive procedures that can facilitate a decrease in the cumulative dosages of corticosteroids and immunosuppressants have great value in the management of PV. CO2 laser vaporization is a safe procedure with minimal morbidity and no long-term side effects and should be considered as an adjunctive treatment option for management of recalcitrant lesions in patients with oral PV.
Source: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology - Category: ENT & OMF Source Type: research
We report 2 clinical cases: The first affected a female patient with erosions and ulcers lesions on the lip and intraoral mucosa who was complaining of severe pain.
Source: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontics - Category: ENT & OMF Authors: Source Type: research
AbstractRituximab (MabThera®, Rituxan®), a chimeric murine/human anti-CD20 monoclonal antibody administered by intravenous infusion, is indicated for the treatment of moderate to severe pemphigus vulgaris (PV), in combination with a tapering course of corticosteroids. Approval in the EU and USA was based on data for the subset of patients with newly-diagnosed, previously untreated PV participating in the randomized, controlled RITUX 3 study; rituximab plus short-course prednisone resulted in a  >  3-fold higher rate of complete remission off prednisone therapy and a >  2-fold decre...
Source: American Journal of Clinical Dermatology - Category: Dermatology Source Type: research
Pemphigus foliaceus is an autoimmune disease that is sporadic around the world but endemic in Brazil, where it is known as fogo selvagem (FS). Characterized by autoantibodies against the desmosomal cadherin desmoglein 1, FS causes painful erosions, and crusts that may be widespread. The recognition of antigens, including exposed sugar moieties, activates the complement system. Complement receptor 1 (CR1, CD35), which is responsible for the Knops blood group on erythrocytes (York and McCoy antigens), is also expressed by antigen-presenting cells. This regulates the complement system by removing opsonized antigens, blocking ...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Authors: Yanovsky RL, McLeod M, Ahmed AR Abstract Introduction: Corticosteroids and immunosuppressive agents have been the mainstay for the treatment of pemphigus vulgaris (PV) for several decades. While these agents have benefited numerous patients, they have been associated with the risks of prolonged immune suppression and have a high incidence of significant and catastrophic side effects. These side effects can be dose limiting. Relapses are frequent. Novel agents promising targeted therapies, that may be provide better outcomes, are being studied. Areas covered: Recently anti-CD20 therapy in combination with c...
Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research
Authors: Bilgic AO, Murrell DO Abstract INTRODUCTION: Pemphigus, an autoimmune disease group characterized by blisters and erosions of the skin and/or mucosal membranes, has been treated with systemic corticosteroids (CS) and immunosuppressive therapies for the past few decades. Areas Covered: However, common adverse effects and complications of long-term CS and immunosuppressive drugs are limiting their long-term use. The disease results in death if not treated. Thus, currently, researchers are trying to develop new and safer therapeutic approaches. Specifically, targeted therapies to pathogenic immune pathways ar...
Source: Expert Review of Clinical Pharmacology - Category: Drugs & Pharmacology Tags: Expert Rev Clin Pharmacol Source Type: research
Publication date: 7–13 September 2019Source: The Lancet, Volume 394, Issue 10201Author(s): Enno Schmidt, Michael Kasperkiewicz, Pascal JolySummaryPemphigus consists of a group of rare and severe autoimmune blistering diseases mediated by pathogenic autoantibodies mainly directed against two desmosomal adhesion proteins, desmoglein (Dsg)1 and Dsg3 (also known as DG1 and DG3), which are present in the skin and surface-close mucosae. The binding of autoantibodies to Dsg proteins induces a separation of neighbouring keratinocytes, in a process known as acantholysis. The two main pemphigus variants are pemphigus vulgaris,...
Source: The Lancet - Category: General Medicine Source Type: research
CONCLUSIONS: In patients with moderate to severe PV, rituximab plus short-term prednisone was more effective than prednisone alone. Rituximab-treated patients had less CS exposure and were less likely to experience severe or life-threatening CS-related AEs. This article is protected by copyright. All rights reserved. PMID: 31487383 [PubMed - as supplied by publisher]
Source: The British Journal of Dermatology - Category: Dermatology Authors: Tags: Br J Dermatol Source Type: research
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