Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: A cohort analysis of 94 elderly patients
Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: A cohort analysis of 94 elderly patients
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DOI：
10.1111/j.1365-2133.1997.tb03654.x
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发表时间：
1997-05-01
影响因子：
10.3
通讯作者：
Bonnetblanc, JM
中科院分区：
文献类型：
--
作者：
Bernard, P;Bedane, C;Bonnetblanc, JM
大疱性类天疱疮（BP）是一种通常影响老年患者的疾病，其预后尚未得到很好的确定，有关该疾病死亡率的报道数据相互矛盾。本研究的目的是评估94例BP患者的临床和免疫因素对生存率的影响。在一个科室连续研究了94例BP患者（平均年龄+/- sd: 81 +/- 4岁），随访时间为8年（1987- 1994），随访时间至少为1年。BP的诊断依据临床标准（使用标准化问卷）、直接免疫荧光（IF）结果（即IgG和/或C3沿基底膜区呈线性沉积），并通过直接免疫电镜和/或免疫印迹法确认。我们的分析（中位随访时间：5年）显示37%的BP患者在开始治疗的一年内死亡。以治疗一年后死亡或存活为标准，研究可能影响BP预后的临床或免疫因素。以下因素均未发现与BP预后显著相关：年龄、性别、出现时皮肤病变的程度、粘膜病变的存在、血嗜酸性粒细胞增多或间接IF引起的循环基底膜区自身抗体的存在。一般情况受损和冠状动脉病史提示预后不良。表皮提取物免疫印迹检测发现，在治疗一年内死亡的BP患者中，循环中存在针对BP180自身抗原的自身抗体，而不存在针对BP230的自身抗体（60% vs. 25%） （P < 0.01）。我们的结论是，循环中针对BP180的自身抗体的存在是BP的第一个内在预后因素。这一结果支持了越来越多的证据，证明抗bp180自身抗体在病理生理上的重要性。
The prognosis of bullous pemphigoid (BP), a disorder which usually affects elderly patients, is not well established and conflicting data have been reported about the mortality rate of the disease. Our objective in this study was to assess the clinical and immunological factors determining survival in a prospective series of 94 patients with BP. A cohort of 94 consecutive patients with BP (mean age +/-SD: 81 +/- 4 years) was studied over an 8-year period (1987-94) in one department and patients followed up for at least 1 year. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (i.e. linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting. Our analysis (median duration of follow-up: 5 years) showed that 37% of BP patients were dead within a year of starting treatment. The clinical or immunological factors which may influence the prognosis of BP were studied according to the criterion of death or survival by the end of the first year of treatment. None of the following factors was found to be significantly linked to the prognosis in BP:age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, or the presence of circulating basement membrane zone autoantibodies by indirect IF. An impaired general condition and a history of coronary artery disease indicated a bad prognosis. The presence of circulating autoantibodies against BP180 autoantigen but not autoantibodies against BP230, as detected by immunoblotting on epidermal extracts, was found to be significantly more frequent (60% vs. 25%) in BP patients who died within the first year of treatment (P < 0.01). We conclude that the presence of circulating autoantibodies against BP180 represents the first intrinsic prognostic factor that has been demonstrated in BP. This result supports the growing body of evidence for the pathophysiological importance of the anti-BP180 autoantibodies.