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Objective To analyze the diagnosis and therapy of pemphigus and pemphigoid.Methods Clinical data were retrospectively analysed for 648 patients with pemphigus or pemphigoid admitted in Huashan Hospital,Fudan University from 2003 to 2007.Results The mean age of onset of pemphigus was younger than that of pemphigoid(47.0±16.9 years vs 65.1±13.9 years.P＜0.001).Of 175 patients with pemphigus confirmed by direct immunoflorescence(DIF),100%were positive for IgG,and 92.0%for C3,while out of 223 patients with pemphigoid,51.12%were positive for IgG,99.1%for C3.For pemphigus,the consistency reached 68.8%among clinical diagnosis.pathological diagnosis and immunological(DIF)diagnosis,80.7%between pathological and immunological diagnosis.In the case of pemphigoid,the consistency was 62.8%among clinical,pathological and immunological diagnosis,and 78.1%between pathological and immunological diagnosis.Corticosteroids were primary treatment strategy for both pemphigus and pemphigoid.and prednisone of 0.5-1.5 mg per kilogram bodyweight per day could control the condition of most patients.Conclusions DIF could be used as an important diagnostic means for patients with pemphigus or pemphigoid which can not be confirmed by clinical manifestation or pathology.In primary hospitals,IgG and C3 are recommended for DIF testing when pemphigus is suspected,and C3 is recommended for suspected pemphigoid.
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Objective To investigate the association between HLA classⅡgenes and bullous pem-phigoid(BP).Methods HLA-DRB1,DQA1,DQB1were determined by polymerase chain reaction se-quence specific oligonucleotide probe methods in56patients with BP and150normal controls from Shanghai area.Results It was shown that HLA-DRB1*10(*1001)was linked to DQB1*0501,and its frequency in-creased significantly in BP group compared to normal controls.There was a positive correlation between DRB1*1001and mucosal involvememt as well as autoantigen BP230.HLA-DRB1*04was linked to DQB1*0302,and its frequency increased significantly in BP group compared with normal control.There was a positive correlaton between DRB1*04and autoantigen BP180.The frequency of HLA-DRB1*12(*1201,*1202)decreased in BP group compared to normal controls.Conclusions It is suggested that HLA-DRB1*1001and DRB1*04be the susceptible genes while DRB1*1201,and*1202be the protective genes of BP patients in Shanghai area.
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PurposeTo compare measures of coronary flow reserve by an intracoronary Doppler guide wire with results of dipyridamole emission computed tomographic 201T1/ 99m Tc-sestamibi myocardial imaging(SPECT).MethodsThe coronary blood flow velocity were measured by Cardiometrics Flomap Ⅱ and 0. 014 or 0. 018 inches Doppler flowire in 27 patients (55 arteries) with coronary angiography. 8 patients (19 arteries )without underlying diseases of coronary microvascular lesions were studied as the normal control. The proximal and distal average peak velocity (APV), diastolic/systolic velocity ratio (DSVR), CFR and proximal/distal velocity ratio (P/DVR) were measured. The results were compared with that of SPECT before angiography. ResultsAccording to the CFR measured by Doppler fiowire, the arteries were divided into two groups:Group A, consisted of 33 arteries with CFR > 2.0. Group B consistedof 22 arteries with CFR≤2.0. CFR of group A was significantly higher than that of group B (P< 10 - 7). Among 22 arteries with abnormal CFR, SPECT could detect 16. Among 33 arteries with normal CFR, all arteries except 2 showed normal SPECT. With intracoronary Doppler flowire as reference, the sensitivity and specificity of dipyridamole SPECT were 72.73 % and 93.94 %. With results of dipyridamole SPECT as reference, the sensitivity and specificity of intracoronary Doppler CFR were 88.89% and 83.78%. Their conformability rate was 85.45%.Conclusions In evaluating coronary reserve function, results of dipyridamole SPECT was rather conformable to measures of intracoronary Doppler CFR, but they cannot be replaced each other.
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Objective To probe the Th cytokine pattern in pemphigus,so as to clarify the pathogenesis of pemphigus and draw up new therapy.Methods Reverse transcription-polymerase chain reaction(RT-PCR)was used to examine the interferon gamma(IFN-?)and interleukin10(IL-10)mRNA expression on peripheral blood mononuclear cells(PBMCs)of20cases of pemphigus in acute stage,20cases in stable stage,and20normal controls.Results The IFN-?mRNA expression in20cases of pemphigus in acute stage was higher than that of controls(P
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0.05). The screening tests for LA is simple and reproducible and it may be useful in clinical study of SLE.
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Objective To study serum levels of interleukin-4,10,interferon-g amma,tumor necrosis factor alpha and soluble interleukin-2 receptor in patients with pemphigus, and their correlation with disease severity. Methods Serum leve ls of different cytokines were determined by double antibody sandwich enzyme-li nked immunosorbent assay in 31 patients with pemphigus in acute and remission st ages, and in 15 normal controls. Serum autoantibody titer was determined by indi rect immunofluorescent assay (IIF). The severity of disease was recorded by the Rule of Nine for the calculation of burn surface area. Results IL-10 level was lower in the sera of patients in their acute stage than that of controls(P0.05). Conclusion There are abnormalities of serum levels of cytokine s in patients with pemphigus. Serum levels of IL-10, sIL-2R and IFN-?are cor related with the severity of the disease.
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Using immunoblotting analysis and salt-split skin indirect immunofluorescence, we studied sera from 5 patients with bullous systemic lupus erythematosus (BSLE) and 55 control subjects,including 20 cases of SLE, 20 cases of bullous pemphigoid (BP),5 cases of epidermolysis bullosa acquisita (EBA) and 10 normal volunteers. The results showed that the sera from 3 of 5 BSLE patients combined with dermal side of saltsplit skin and 290 000 antigen of dermal extracts in human skin. Sera from 2 BSLE patients which reacted with 290 000 antigen also combined with 165 000 antigen of epidermal extracts. Sera from SLE patients did not combined with both 290 000 and 165 000 antigens. The results suggest that there are EBA antibody and BP antibody in BSLE. We consider that EBA antigen and BP antigen may be the related antigens of skin basement membrane zone in BSLE.