Department of Dermatology, Reims University Hospital, University of Champagne-Ardenne, Reims, France.
Clinical Research Unit, Reims University Hospital, Reims, France.
JAMA Dermatol. 2014 Jan;150(1):25-33. doi: 10.1001/jamadermatol.2013.5757.
Although predisposing factors for bullous pemphigoid (BP) have been recently established, no clinical or immunologic factors have yet been identified to predict disease outcome.
To identify risk factors for BP relapse during the first year of treatment.
DESIGN, SETTING, AND PARTICIPANTS: Multicenter prospective study of 120 consecutive patients with newly diagnosed BP in 8 French dermatology departments. Baseline and 6 follow-up visits were planned to record disease activity and collect blood samples for measurement of serum anti-BP180 and anti-BP230 levels by means of enzyme-linked immunosorbent assay (ELISA).
The end point was clinical relapse within the first year of therapy. Associations of clinical and immunologic (including serum levels of anti-BP180 and anti-BP230 autoantibodies) parameters with clinical relapse were assessed using univariate and multivariate analyses.
During the 1-year follow-up, 35 patients (29.2%) experienced relapse, whereas anti-BP180 and anti-BP230 ELISA results were similar at baseline between patients who did and did not experience relapse. Factors at baseline independently associated with relapse were extensive disease at inclusion (hazard ratio [HR], 2.37 [95% CI, 1.2-4.8]) and an associated dementia (HR, 2.09 [95% CI, 1.0-4.2]). Use of superpotent topical corticosteroids alone (by 100 patients [83.3%]) induced a dramatic, early decrease in serum levels of anti-BP180 and anti-BP230 autoantibodies. Mean early decreases in autoantibody levels between baseline and day 60 were lower in patients with relapse compared with patients with ongoing remission (-10.0% and -45.2%, respectively, for anti-BP180 levels [P < .001] and -11.8% and -35.4%, respectively, for anti-BP230 levels [P = .046]). A higher serum level of anti-BP180 at day 150, with a cutoff of 23 U/mL, provided 84.2% sensitivity, 44.8% specificity, 33.3% positive predictive value, and 89.7% negative predictive value for the occurrence of relapses between days 150 and 360.
The pronounced decrease in the level of anti-BP180 autoantibodies and, to a lesser extent, those directed against BP230 confirmed the use of superpotent topical corticosteroids alone as a reference BP treatment. Furthermore, our study suggests that neurological diseases play a major role in BP, not only as a predisposing but also as a prognostic factor.
尽管最近已经确定了大疱性类天疱疮（BP）的易患因素，但尚未发现任何临床或免疫因素可预测疾病结局。
确定治疗开始后第一年 BP 复发的风险因素。
设计、地点和参与者：在法国 8 个皮肤科部门对 120 例新诊断为 BP 的连续患者进行了多中心前瞻性研究。计划进行基线和 6 次随访，以记录疾病活动并采集血液样本，通过酶联免疫吸附测定（ELISA）测量血清抗 BP180 和抗 BP230 水平。
终点是治疗开始后 1 年内的临床复发。使用单变量和多变量分析评估临床和免疫（包括血清抗 BP180 和抗 BP230 自身抗体水平）参数与临床复发的关联。
在 1 年的随访期间，35 名患者（29.2%）出现复发，而在出现和未出现复发的患者中，基线时抗 BP180 和抗 BP230 ELISA 结果相似。与复发相关的基线独立因素包括纳入时广泛的疾病（危险比[HR]，2.37 [95%CI，1.2-4.8]）和相关痴呆（HR，2.09 [95%CI，1.0-4.2]）。单独使用超强效外用皮质类固醇（由 100 名患者[83.3%]使用）可显著早期降低血清抗 BP180 和抗 BP230 自身抗体水平。与持续缓解的患者相比，复发患者在基线至第 60 天之间的自身抗体水平早期下降幅度较低（抗 BP180 水平分别为-10.0%和-45.2%[P<0.001]，抗 BP230 水平分别为-11.8%和-35.4%[P=0.046]）。第 150 天血清抗 BP180 水平较高，截断值为 23 U/mL，可提供 150 至 360 天之间发生复发的 84.2%的敏感性、44.8%的特异性、33.3%的阳性预测值和 89.7%的阴性预测值。
抗 BP180 自身抗体水平的明显下降，以及对 BP230 的抗体水平下降在较小程度上证实了单独使用超强效外用皮质类固醇是 BP 的标准治疗方法。此外，我们的研究表明，神经疾病不仅是一种易患因素，也是一种预后因素，在 BP 中起着重要作用。
