Monshi B, Gulz L, Piringer B, Wiala A, Kivaranovic D, Schmidt M, Sesti A, Heil T, Vujic I, Posch C, Rappersberger K
Department of Dermatology and Venereology, The Rudolfstiftung Hospital, Vienna, Austria.
Department of Neonatology, Johannes Kepler University Linz, Med Campus IV, Linz, Austria.
J Eur Acad Dermatol Venereol. 2020 Jul;34(7):1583-1589. doi: 10.1111/jdv.16363. Epub 2020 Jul 2.
Bullous pemphigoid (BP) is the most frequent autoimmune blistering disease mainly affecting elderly patients. Among several published risk factors, a recent post hoc analysis linked anti-BP180 autoantibodies (AABs) to fatal outcomes in BP. To date, this finding has not been confirmed independently.
To investigate the potential of anti-BP180-AAB levels as a marker of prognosis and to identify a cut-off level indicative of an increased risk for early death. Secondly, to characterize parameters associated with mortality.
Retrospective, single-centre study of BP patients diagnosed between 2001 and 2012. Analyses included epidemiological and patient- and disease-specific characteristics as well as immunological parameters at diagnosis and during follow-up. Standardized mortality ratios as well as uni- and multivariate regression analyses were calculated.
One hundred patients (56 women, 44 men) with a median age of 81 years (interquartile range 74-86) were followed up for a median of 775 days (interquartile range 162-1617). One-year mortality rates were 25.0% implying a 2.4-fold increased risk of death compared with the general population. High anti-BP180 autoantibody levels at diagnosis (CI 1.30-2.89; P = 0.001), dementia (CI 1.13-6.72; P =0.03), length of hospitalization (CI 1.16-2.41; P = 0.01) and age (CI 1.23-4.19; P = 0.009) correlated significantly with 1-year mortality. BP180-AAB concentrations of ≥61 U/mL characterized a subgroup of patients with a particular higher risk for early death compared with the general population (CI 1.81-3.81; P < 0.0001).
In bullous pemphigoid, serum concentrations of BP180 autoantibodies at diagnosis could help to identify patients at risk for death within the first year after diagnosis (cut-off value 61 U/mL).
大疱性类天疱疮（BP）是最常见的自身免疫性水疱病，主要影响老年患者。在已公布的多个风险因素中，最近一项事后分析将抗BP180自身抗体（AABs）与BP的致命结局联系起来。迄今为止，这一发现尚未得到独立证实。
研究抗BP180-AAB水平作为预后标志物的潜力，并确定一个表明早期死亡风险增加的临界值。其次，对与死亡率相关的参数进行特征描述。
对2001年至2012年间诊断为BP的患者进行回顾性单中心研究。分析内容包括流行病学、患者及疾病特异性特征，以及诊断时和随访期间的免疫学参数。计算标准化死亡率以及单因素和多因素回归分析。
100例患者（56例女性，44例男性），中位年龄81岁（四分位间距74-86岁），中位随访时间775天（四分位间距162-1617天）。1年死亡率为25.0%，这意味着与普通人群相比死亡风险增加了2.4倍。诊断时抗BP180自身抗体水平高（可信区间1.30-2.89；P = 0.001）、痴呆（可信区间1.13-6.72；P = 0.03）、住院时间（可信区间1.16-2.41；P = 0.01）和年龄（可信区间1.23-4.19；P = 0.009）与1年死亡率显著相关。与普通人群相比，BP180-AAB浓度≥61 U/mL可确定一组早期死亡风险特别高的患者亚组（可信区间1.81-3.81；P < 0.0001）。
在大疱性类天疱疮中，诊断时BP180自身抗体的血清浓度有助于识别诊断后第一年内有死亡风险的患者（临界值61 U/mL）。
