| Background Bullous pemphigoid(BP)is an immune Bullous disease with high mortality and disability rate.The characteristics of patients in different areas of the world are also different.There are few domestic studies on retrospective analysis and comorbidities spectrum of BP with the data of large sample,and the epidemiological findings show that BP is closely related to Neurological diseases(ND).BP autoantigens BP180 and BP230 are expressed in the basement membrane of the skin and the central nervous system and may be involved in the pathogenesis.Part 1.A retrospective analysis of 390 cases of bullous pemphigoidObjectives To study the demographic and clinical characteristics of bullous pemphigoid in China and provide a reference for its diagnosis and treatmentMethods We retrospectively analyzed 390 patients with bullous pemphigoid admitted to the first affiliated hospital of anhui medical university from 2003 to 2018.The demographic characteristics,clinical characteristics,complications,treatment and laboratory data of the patients were collected and analyzed.Results A total of 390 patients(223 males and 167 females)were included in this study,with an average age of onset of 70.42±13.72 years.There was no significant difference in age of onset between different genders(Z=-1.65,p=0.098).The difference of gender distribution in different age groups was statistically significant(?~2=11.788,p=0.019).There was no statistical significance in the distribution of age components of patients of different genders(?~2=6.528,p=0.163).In this study,all patients presented with typical vesicle and bullae,and other common clinical manifestations included pruritus(78.98%),Wheal or urticarial plaque(12.05%),erosion or ulcer(37.95%).13.59%of the patients had lesions that involved oral or genital mucosa.49.74%of the patients had hypoalbuminemia.47.18%of the patients showed elevated eosinophil in their peripheral blood.The positive rate of Histopathologic examination,the direct immunofluorescence,indirect immunofluorescence and BP180antibody were 97.98%,90.23%,66.19%and 82.33%respectively.Among the BP complications,cardiovascular diseases were the most common(43.85%),among which hypertension accounted for 32.05%,other heart diseases accounted for 11.79%.Neurological diseases were the second most common(24.68%),among which stroke was the most common(20.26%),followed by Parkinson's disease(3.33%),dementia(1.28%),epilepsy(1.28%),brain tumor(1.02%).Diabetes(13.37%),lung diseases(9.49%),tumor(5.64%),nephropathy(3.59%),rheumatoid disease(3.33%)and psoriasis(1.79%)are also common comorbidity of BP.All patients in this study received topical corticosteroids.9 patients(2.31%)were given adequate topical corticosteroids alone.96.92%of the patients received systematic corticosteroids,including 20 patients(5.23%)were added azathioprine and 48 patients(12.31%)were added intravenous immunoglobulin.Two patients(0.51%)did not receive systemic glucocorticoid,one case(0.26%)received intravenous immunoglobulin alone,and another case(0.51%)received azathioprine alone.Conclusions BP often affects the elderly,and there is no gender difference in age of onset and age composition between men and women.There are gender differences in different age groups.The majority of cases occurred in women under the age of 50,while the majority occurred in men over the age of 50.BP patients are often diagnosed with a variety of diseases,among which cardiovascular disease and nervous system disease are the most common.Neurological diseases were diagnosed 7.99±9.65 years before BP on average.Direct immunofluorescence,indirect immunofluorescence and BP180 have high sensitivity as auxiliary diagnostic methods of BP.Corticosteroids are still the primary treatment for BP and showed significanteffect with immunosuppressant and other drugs.Part 2.The analysis of the autoimmune response against BP180and BP230 with neurological diseases and bullous pemphigoidObjectives To analyze the serological immunopathological profiles(anti-BP180 Ig G,anti-BP230 Ig G)in BP patients with and without ND,ND patients and normal person,and compare eosinophil level in peripheral blood,the dosage of corticosteroids in order to identify the specific autoantibody and corresponding antigens responsible for ND development in BP patients.Methods Altogether,145 patientsand their medical records were examined(43BP-ND,21 BP+ND,41 ND,40 normal control).Levels of serum anti-BP180/BP230 Ig G in 4 groups were evaluated with ELISA and The dates of eosinophil level in peripheral blood and the dosage of corticosteroids were collected to compare the differences between groups.Results The majority occurred in men in BP-ND and BP+ND group,and there was no difference in gender distribution(?~2=0.190,p=0.663).The age difference between BP-ND group and BP+ND group was statistically significant(t=2.308,p=0.026).The positive rates and antibody levels of BP180 and BP230 in the BP-ND group and the BP+ND group were both higher than those in the ND group and the normal control group(All P values were<0.05).The positive rates of BP180 and BP230antibodies(?~2=0.820,p=0.365)(?~2=3.472,p=0.062)and antibody levels(t=1.060,p=0.293)the BP-ND group and the BP+ND group were not statistically different.The positive rate and antibody level of BP180 and BP230 in ND group and normal control group were not significantly different(All P values were>0.05).There was no significant difference in the probability of ND in BP patients with different qualitative detection results of BP antibody(All P values were>0.05).The levels of eosinophil in peripheral blood of the BP-ND group and the BP+ND group showed no statistically difference(?~2=0.195,p=0.659),and the differencethe dosage of corticosteroids between 2 groups were statistically significant(t=2.079,p=0.043).Conclusions The age of onset of BP+ND group was higher than that of BP-ND group.BP patients with ND need larger doses of corticosteroids to control the disease.Pathogenetic association of BP and ND appears to be independent of a particular BP antigen and eosinophil in peripheral blood.The qualitative detection results of BP antibody were not associated with ND... |
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