Department of Dermatology, Counties Manukau Health, Auckland, New Zealand.
Department of Dermatology, Auckland District Health Board, Auckland, New Zealand.
Australas J Dermatol. 2022 May;63(2):197-203. doi: 10.1111/ajd.13822. Epub 2022 Mar 24.
BACKGROUND/OBJECTIVES: Bullous pemphigoid (BP) is an uncommon autoimmune bullous disorder, with significant morbidity and mortality. Mortality may be as high as 23.5% in the first year after diagnosis. Clear epidemiologic data across Australasia are lacking.
A retrospective, multi-centred cohort study was designed to determine the incidence and mortality of bullous pemphigoid in New Zealand. Data from all histopathologically diagnosed patients with bullous pemphigoid between 2009 and 2015 from the Auckland region were obtained. Demographics, clinical characteristics and outcome 3 years from diagnosis (until 31 December 2018) were collected. Demographic data were compared against a denominator year-matched New Zealand Census population.
One hundred sixty-one patients had confirmed bullous pemphigoid, with an incidence rate of 3.03/100 000 person-years [95% CI 2.58-3.54]; 70% were of European ethnicity; 12.4% were Pacific peoples; 11.2% were Asian; and 6.8% were Māori. 45.3% had associated cognitive impairment and/or stroke. In the 3-year follow-up, 25% had treatment complications mostly from prednisone therapy. The mortality rate was 40%, highest in the first year of diagnosis, with age at diagnosis a predictor.
The incidence and mortality rates are comparable to the UK/Northern Europe. Knowledge of the epidemiology of bullous pemphigoid in New Zealand and within an international settling informs the provision of future care and treatments.
背景/目的：大疱性类天疱疮（BP）是一种罕见的自身免疫性大疱性疾病，具有较高的发病率和死亡率。在诊断后的第一年，死亡率可能高达 23.5%。目前在整个澳大拉西亚地区缺乏明确的流行病学数据。
本研究采用回顾性、多中心队列研究设计，旨在确定新西兰大疱性类天疱疮的发病率和死亡率。收集了 2009 年至 2015 年间奥克兰地区所有经组织病理学诊断为大疱性类天疱疮的患者数据。收集了患者的人口统计学、临床特征和诊断后 3 年（截至 2018 年 12 月 31 日）的结局数据。将人口统计学数据与同年新西兰人口普查的分母进行了比较。
共有 161 例患者确诊为大疱性类天疱疮，发病率为 3.03/100000 人年[95%可信区间 2.58-3.54]；70%为欧洲血统；12.4%为太平洋岛民；11.2%为亚洲人；6.8%为毛利人。45.3%的患者伴有认知障碍和/或中风。在 3 年的随访中，25%的患者出现治疗并发症，主要与泼尼松治疗有关。死亡率为 40%，在诊断后的第一年最高，诊断时的年龄是一个预测因素。
新西兰的发病率和死亡率与英国/北欧相当。了解新西兰和国际移民中大疱性类天疱疮的流行病学情况，为未来的护理和治疗提供了信息。
