Kridin Khalaf, Sagi Shira Zelber, Bergman Reuven
Department of Dermatology, Rambam Health Care Campus. POB 9602, 31096 Haifa, Israel.
Acta Derm Venereol. 2017 May 8;97(5):607-611. doi: 10.2340/00015555-2611.
All-cause and cause-specific mortality among patients with pemphigus compared with the general population is yet to be established. This study investigated overall mortality and cause-specific mortality in a large immunopathologically validated cohort of patients with pemphigus. Mortality of patients with pemphigus was compared with age- and gender-matched control subjects in the general population. All-cause and cause-specific standardized mortality ratios (SMRs) were estimated. The study cohort included 245 patients newly-diagnosed with pemphigus between January 1990 and June 2016, contributing 2,679.4 person-years of follow-up. Overall, 48 deaths were observed during a mean follow-up period of 10.9 ± 8.1 years, which was more than twice the number expected (SMR 2.4; 95% confidence interval (95% CI) 1.82-3.20). The SMRs for death due to infections (22.6; 95% CI 13.6-35.3), namely pneumonia (25.7; 95% CI 11.7-48.8) and septicaemia (8.6; 95% CI 1.7-25.0), and due to cardiovascular diseases (2.8; 95% CI 1.0-6.0) were significantly higher than expected. Overall mortality among patients with pemphigus is 2.4-times greater than for the general population, mainly due to infections.
与普通人群相比，天疱疮患者的全因死亡率和特定病因死亡率尚未确定。本研究调查了一大组经免疫病理学验证的天疱疮患者的总体死亡率和特定病因死亡率。将天疱疮患者的死亡率与普通人群中年龄和性别匹配的对照对象进行比较。估计了全因和特定病因的标准化死亡率（SMR）。研究队列包括1990年1月至2016年6月期间新诊断为天疱疮的245例患者，随访时间共计2679.4人年。总体而言，在平均10.9±8.1年的随访期内观察到48例死亡，这一数字是预期死亡人数的两倍多（SMR 2.4；95%置信区间（95%CI）1.82 - 3.20）。因感染导致的死亡的SMR（22.6；95%CI 13.6 - 35.3），即肺炎（25.7；95%CI 11.7 - 48.8）和败血症（8.6；95%CI 1.7 - 25.0），以及因心血管疾病导致的死亡的SMR（2.8；95%CI 1.0 - 6.0）均显著高于预期。天疱疮患者的总体死亡率比普通人群高2.4倍，主要原因是感染。
