AbstractIn this study, we aimed to examine the relationship between the serum cytokine levels of patients with pemphigus vulgaris (PV) and the Pemphigus Disease Area Index (PDAI), along with the presence of anti‐desmoglein (Dsg) 1 antibody, anti‐Dsg3 antibody and co‐infection among patients with pemphigus vulgaris. This retrospective study included 62 PV patients and 59 healthy individuals who attended the Second Affiliated Hospital of Kunming Medical University from November 2014 to November 2022. The serum concentrations of cytokines and chemokines were assessed using the Luminex 200 System (a high‐throughput cytokine detection method). Additionally, anti‐Dsg1 and anti‐Dsg3 antibodies were determined through enzyme‐linked immunosorbent assay, while disease severity was evaluated using the PDAI scoring system. The PV group exhibited elevated levels of Th1 cytokines (such as interleukin (IL)‐1RA, IL‐1β, IL‐2, IL‐12p70, GM‐CSF, TNF‐α, IL‐18, IFN‐γ), Th2 cytokines (IL‐5, IL‐10, IL‐13) and Th17/Th22‐related cytokines (IL‐17A, IL‐22) compared to the healthy control group (p < 0.05). Conversely, the levels of chemokines (macrophage inflammatory protein‐1 alpha (MIP‐1α), stromal cell‐derived factor‐1 alpha (SDF‐1α), interferon‐inducible protein‐10 (IP‐10), Regulated on Activation in Normal T‐Cell Expressed And Secreted (RANTES), growth‐regulated on‐gene‐alpha (GRO‐α), MIP‐1β) and Th2 (IL‐31) were lower in the PV group compared to the healthy control group (p < 0.05). No significant differences were observed in other cytokines and chemokines (p > 0.05). Additionally, IL‐7, IFN‐γ, IL‐18 and GRO‐α showed positive correlations with PDAI, IL‐6 correlated positively with anti‐Dsg3 antibody levels, and IL‐12p70, IL‐18, and IFN‐γ correlated positively with anti‐Dsg1 antibody levels. Furthermore, IL‐15 exhibited a positive association with skin infections. PV patients have elevated levels of various cytokines and chemokines, and there are different degrees of elevation in cytokines and chemokines associated with the activation of various T cell subsets. PDAI and the Dsg1 antibody levels are mainly related to the Th1‐related cytokines.

2013-02-25·Endocrine Connections3区 · 医学

Absence of some common organ-specific and non-organ-specific autoimmunity in autoimmune polyendocrinopathy candidiasis ectodermal dystrophy

3区 · 医学

ArticleOA

作者: Krohn, Kai ; Ranki, Annamari ; Kluger, Nicolas

BACKGROUNDAutoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED) is a rare autosomal recessive disorder caused by mutations of the autoimmune regulator (AIRE) gene, whose loss of function leads to the escape of self-reactive T cells from the thymus and autoimmunity. APECED patients typically develop tissue-specific autoantibodies and anti-cytokine antibodies. Consequently, various endocrine and non-endocrine autoimmune disorders appear. However, only a certain number of autoimmune diseases develop, while some common autoimmune conditions have not been reported or are seen only anecdotally.OBJECTIVEWe investigated the clinical manifestations and occurrence of antinuclear antibodies (AN-Abs) and antibodies against extractable nuclear antigens, citrullinated peptide, and transglutaminase in 24 patients and against bullous pemphigoid antigen 180 and desmogleins 1 (Dsg1) and Dsg3 in 30 patients of a Finnish cohort of APECED patients.RESULTSDespite the loss of central tolerance, the autoantibodies investigated were not overrepresented among the APECED patients. None of the patients had a history of autoimmune connective tissue disease, rheumatoid arthritis, celiac disease, or autoimmune cutaneous bullous disorders. Altogether, 25% (6/24) had low-titer (1:80) AN-Abs. Two patients had anti-BP180 antibodies and two others had anti-Dsg3 antibodies without any cutaneous or mucosal symptoms. No anti-citrullinated peptide and anti-transglutaminase reactivity was found.CONCLUSIONSThe mechanisms that drives tolerance to tissue autoantigens is not fully understood as even APECED patients, who are genetically prone to develop autoantibodies, are tolerant against some common autoantigens. The hypothesis that some of the anti-cytokine antibodies commonly found in APECED patients may be protective should be investigated in larger series.

1998-04-01·Journal of Investigative Dermatology1区 · 医学

Heterogeneous MHC II Restriction Pattern of Autoreactive Desmoglein 3 Specific T Cell Responses in Pemphigus Vulgaris Patients and Normals

1区 · 医学

Article

作者: Michael Hertl ; Robert W. Karr ; Stephen I. Katz ; Masayuki Amagai

Pemphigus vulgaris is a life threatening bullous autoimmune disease of the skin mediated by autoantibodies against desmoglein 3 (Dsg3) on epidermal keratinocytes. Pemphigus vulgaris patients exhibit T cell responses against Dsg3 that may serve as a target to modulate the production of pathogenic autoantibodies. Healthy carriers of major histocompatibility complex class II alleles identical or similar to those that are highly prevalent in pemphigus vulgaris, namely DRbeta1*0402 and DRbeta1*1401, also mount T cell responses against Dsg3. We thus wanted to determine whether these prevalent major histocompatibility complex class II alleles restricted Dsg3 specific T cell responses. A CD4+ T cell line from the DRbeta1*0402+ patient PV9 was stimulated by Dsg3 with DRbeta1*0402+ L cells as antigen-presenting cells. A CD4+ T cell line and six CD4+ T cell clones from the DR11/14+ patient PV8, and six CD4+ T cell clones from the DR11+ healthy donor C6, required DR11/ DQbeta1*0301+ peripheral blood mononuclear cells but not DR11+ L cells as antigen-presenting cells and were strongly inhibited by anti-DQ antibodies, indicating that they were restricted by HLA-DQbeta1*0301. A CD4+ T cell line and three T cell clones from the DR11+ healthy donor C11 were differentially stimulated by Dsg3 with L cells expressing one of several DR11 alleles. T cell recognition of Dsg3 was thus not only restricted by the pemphigus vulgaris associated DRbeta1*0402 allele, but also by several DR11 alleles, some of which are highly homologous to DRbeta1*0402, and by HLA-DQbeta1*0301.

100 项与 DSG3 inhibitors(Chang Gung University) 相关的药物交易

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