Rzany Berthold, Partscht Karin, Jung Martin, Kippes Werner, Mecking Dieter, Baima Bozena, Prudlo Christin, Pawelczyk Beata, Messmer Elisabeth M, Schuhmann Monika, Sinkgraven Ronald, Büchner Lutz, Büdinger Lioba, Pfeiffer Christine, Sticherling Michael, Hertl Michael, Kaiser Hans-Wilhelm, Meurer Michael, Zillikens Detlef, Messer Gerald
Department of Dermatology, Fakultät für Klinische Medizin Mannheim der Universität Heidelberg, Mannheim, Germany.
Arch Dermatol. 2002 Jul;138(7):903-8. doi: 10.1001/archderm.138.7.903.
Although bullous pemphigoid (BP) is the most frequent autoimmune bullous disease and is associated with a considerable case-fatality rate, little is known about factors that influence its prognosis.
To identify prognostic factors for lethal outcome in the first year after the initial hospitalization in patients with BP.
A multicenter retrospective cohort study.
Seven dermatologic university hospitals in Germany.
A total of 369 patients diagnosed as having BP between January 1, 1987, and December 31, 1997.
Univariate (Kaplan-Meier) and multivariate (Cox regression) analysis.
Of the 369 patients with BP, 209 (57%) died, 106 (29%) within the first year after hospitalization. Fifty-four percent were women. The mean +/- SD age at entry was 77.3 +/- 11.1 years. The patients with BP were followed up to 10.5 years, with a median time of 1.8 years to death or interview (25th and 75th quartiles, 0.5 and 4.0 years). The major risk factors for lethal outcome in the first year after hospitalization were an increased age, with a multivariate risk estimate of 3.2 (95% confidence interval [CI], 1.9-5.2) for age greater than 80.4 years (median); a daily glucocorticosteroid dosage of more than 37 mg (75th quartile) at discharge, with a multivariate risk estimate of 2.5 (95% CI, 1.5-4.3); serum albumin levels of 3.6 g/dL or less (25th quartile), with a multivariate risk estimate of 2.6 (95% CI, 1.5-4.4); and an erythrocyte sedimentation rate greater than 30 mm/h (75th quartile), with a multivariate risk estimate of 1.7 (95% CI, 1.1-2.8).
There is a considerable case-fatality rate in patients with BP. Older patients who require a higher dosage of oral glucocorticosteroids at hospital discharge and who have low serum albumin levels are at greater risk of death within the first year after hospitalization. These prognostic factors should be considered in the care of patients with BP as well as in the design of future clinical trials.
尽管大疱性类天疱疮（BP）是最常见的自身免疫性大疱性疾病，且具有相当高的病死率，但对于影响其预后的因素却知之甚少。
确定BP患者首次住院后第一年致死结局的预后因素。
一项多中心回顾性队列研究。
德国的7家大学皮肤科医院。
1987年1月1日至1997年12月31日期间共369例诊断为BP的患者。
单因素（Kaplan-Meier）和多因素（Cox回归）分析。
369例BP患者中，209例（57%）死亡，106例（29%）在住院后第一年内死亡。患者中54%为女性。入院时的平均年龄±标准差为77.3±11.1岁。对BP患者随访长达10.5年，死亡或接受访谈的中位时间为1.8年（第25和第75百分位数分别为0.5年和4.0年）。住院后第一年内致死结局的主要危险因素包括：年龄增加，年龄大于80.4岁（中位数）时多因素风险估计值为3.2（95%置信区间[CI]，1.9 - 5.2）；出院时每日糖皮质激素剂量超过37 mg（第75百分位数），多因素风险估计值为2.5（95% CI，1.5 - 4.3）；血清白蛋白水平为3.6 g/dL或更低（第25百分位数），多因素风险估计值为2.6（95% CI，1.5 - 4.4）；红细胞沉降率大于30 mm/h（第75百分位数），多因素风险估计值为1.7（95% CI，1.1 - 2.8）。
BP患者有相当高的病死率。出院时需要更高剂量口服糖皮质激素且血清白蛋白水平低的老年患者，在住院后第一年内死亡风险更高。在BP患者的治疗以及未来临床试验设计中应考虑这些预后因素。
