The Study Of The Correlation Between Pemphigus Antibody Titer,the Disease Activity And CD19,CD20

Pemphigus is a mainly autoantibody-mediated organ-specific autoimmune diseases. A common clinical feature is the formation of the blisters, or bulla, which is thin-walled,loosing and easy to rupture. Histopathology is at the character of the intraepidermal blister formation caused by acantholytic. Indirect immunofluores-cence,IIF) shows that IgG and C3 deposition between cells. The incidence is low, but it is growing up. It is more common in middle-aged crowd, especially in 30-50 years of age, and it is noisignificant differences in gender.Clinically it can be divided into four types:pemphigus vulgaris(PV), pemphigus vegetans, pemphigus foliaceus(PF), pemphigus erythematosus. The four types of pemphigus can change into each other. Inaddition,there are herpes-like pemphigus, IgA pemphigus, drug-induced pemphigus, paraneoplastic pemphigus, pemphigus pandemic (also known as Brazil pemphigus).Its etiopathogenisis and pathogenesy is not clear so far, Nowadays more and more researchers believe that in the pathogenesis of pemphigus, T cells and B-cell interact and influence each other, producing antibodies to their own materials, then leading to the disease. The antibodies are mainly IgG, there are also IgM and IgA, but more rare than IgG.The composition of the desmosomes are the mian antigens of pemphigus, desmosomes is the main connective structure between keratinocytes, it is shaped by the adjacent cell membrane ovally thicken,and constituted by the two types of protein:one is the transmembrane protein, locating in the desmosomal core, which is mainly composed of desmoglein and desmocollin, they shape the desmosomes e-clear cell and the contact layer between the cell gap; another is the desmosomal plaque protein within cytoplasm, which is a part of the discoid attachment plate.the main components are desmoplakin and plakogloubin.Pemphigus antibody bind to antigen, the cell signaling pathway activated by a series of proteolytic enzymes, leading to hydrolysis of link structure between cells, then leading to epidermal acantholysis cells separated and acantholysis and intraepidermal blister formation. Cadherin is a Ca2+ dependent cell junctive molecule, it iplays an important role in epidermal cell adhesion. There are significantly homologous between the cDNA sequences of pemphigu antigen and E-cadherin, therefore pemphigus antibodies can damag the adhesion function between epidermal cell, leading to acantholytic.Acantholysis is superficial in the PF,the damage only involve in epidermal granular layer.while In the PV,it is between the epidermal germinal layer and the grass-roots level. In almost all pemphigus patients,the immune pathology showes there are IgG and C3 deposition inter-reticular of keratinocytes. The diagnosis of pemphigus based on the following four factors:1,the typical clinical performance of skin lesions; 2, acantholysis in pathological of skin tissue; 3,IgG and C3 deposition between the reticular as showed in the immune pathological; 4, there exist antibodies to its own desmosomal core glycoprotein.that in the serum or in situ of lesions of the patients. Nepal's sign for the diagnosis of pemphigus is high specificity, but poor sensitivity. Direct immunofluorescence (DIF) is the gold standard for diagnosis of pemphigus, but the repeated skin biopsy may cause great suffering to patients, it is difficult for the majority of patients to accept. On the basis of the pathogenesis of pemphigus we have known and the lever of the pemphigus antibodies parallel with the severity of disease, this study attempts to detect circulating pemphigus antibodies to diagnose the disease, and this method is convenient, safe and caused the patient less pain.At present,there are a lot of treatments for patients with pemphigus, but the first choice is still corticosteroids,which start at a large dose to control the formation of new blisters in 1-2 weeks,if necessary, the does of corticosteroids can be increased for impulse therapy or immunosuppressive agents are combinated. According to the lesion area,patient's condition can be divided into mild, moderate and severe. The patients are given appropriate doses of corticosteroids at the criterion of no new blisters forming. Mild means Lesion area account for below 10% of body surface area, and the does of corticosteroids is below 30mg/d; between 30% and 50% of body surface area,namely moderate, corticosteroids amount to 60-80 mg/d; when 50% of body surface area is severe, corticosteroids amount of above 80 mg/d. In addition, some pemphigus patients with very limited lesion area can take a small amount of corticosteroids,at the same time,tacrolimus ointment ointment for external use can promote the healing of skin lesions.However, the biggest problems are the clear diagnosis and determine of the disease activity of pemphigus and prognosis. The specificity of Nepal's sign for the diagnosis of pemphigus is high, but lower sensitivity. DIF examination is the gold standard for diagnosis of pemphigus, but repeated skin biopsy causes more pain for the patients, it is difficult for the majority of patients to accept. It is not a better standard of follow-up. According to the pathogenesis of pemphigus we have known, this study attempts to detect circulating pemphigus antibody to diagnose the disease and the relationship between disease activity and the pemphigus antibody titer to find out the appropriate follow-up indicators of the clinical application.Objection:1.To detect the lever of the pemphigus antibody in the serum of the patients with pemphigus, by enzyme-linked immunosorbent assay (ELISA),and suggesting the sensitivity and specificity in the clinical diagnosis of the disease.2.To rate the degree of disease activity in patients with pemphigus and explore its consistency with the pemphigus antibody titers, analyzing the correlation of pemphigus antibody titers with disease activity.,then drawing scatter and linear and obtaining the regression equation.3.To detect the percentage of CD19 and CD20 in serum of the patient with pemphigus, analyzing the correlation of pemphigus antibody titers with CD 19, CD20 levels,then obtaining the regression equation.Metheod:1.collecting the outpatients and inpatients 38 in all diagnosed with pemphigus and 22 patients with non-pemphigus from Jan,2009 to Feb,2010,detecting.the pemphigus antibody titer in serum of the 60 patient by ELISA, based on the clinical diagnosis to Judge the sensitivity, specificity, and conformance with the clinical diagnosis of the serological diagnosis.2.Confirming pemphigus disease activity rating criteria by Reference, scoring the patients'degree of disease activity, and making the sample correlation analysis between the disease activity score and pemphigus antibody titers, drawing scatter and making the linear regression equation.3.We collected the serum of 28 patients with pemphigus in 38, and detected CD 19, CD20 content in the serum by flow cytometry. The make the multiple correlation analysis between them and the pemphigus antibody titers to the corresponding, and produces a multiple regression equation. 4, Making the sample correlation analysis between the disease activity score and pemphigus antibody titers, drawing scatter and making the linear regression equation. And multiple correlation analysis between them and the pemphigus antibody titers to the corresponding, and produces a multiple regression equation.by SPSS 13.0 statistical analysis software.Result:1,the consistent of pemphigus antibody titers with the clinical diagnosis there were 37 serun positive by ELISA in 38 patients with a clinical diagnosis of pemphigus, one negative.22 serum were negative in patients with non-pemphigus. The sensitivity was 97.4%, and specificity was 100%, and diagnostic accuracy rate was 98.3%.suggesting Sensitivity, specificity and diagnostic accuracy rates were high.2,Pearson bivariate correlation analysis showed that the correlation between disease activity with pemphigus antibody titers are statistically significant (P<0.001), correlation coefficient r=0.671,and the linear regression equation was: score=1.079+0.671* antibody titers. Tip the higher the antibody titer, the stronger the activity of the disease.3,Multiple regression analysis showed that the correlation between pemphigus antibody titers with CD19, CD20 in serum of the pemphigus were also statistically significant (P<0.05), conversing the data to log, its multiple correlation coefficient R =0.55,R2=0.302, the partial regression coefficient of 1gCD19(x1)was 0.518,and the partial regression coefficient of 1gCD20 (x2) was 0.066, and the intercept was 1.674, and obtaining the regression equation:Lgantibody titers=1.674+0.518*LgC-D19+0.066*LgCD20。Conclusion:1,pemphigus antibodies can diagnose pemphigus with high sensitivity and specificity clinically, and it is consistent with clinical diagnosis a higher rate,It has important significance in diagnosis.2, pemphigus antibody titers parallel with disease activity, and with a high correlation. As the antibody titer increased, the stronger the degree of disease activity.3,pemphigus antibody titers is correlated with the CD 19, CD20 content in the serum of patients, CD 19, CD20, with the more content, the antibody titers increased.