Department of Dermatology and Immunodermatology, Medical University of Warsaw, Koszykowa 82a Str, 02-008, Warsaw, Poland.
Human Nutrition Department Faculty of Health Science, Jagiellonian University Medical College, Kraków, Poland.
Arch Dermatol Res. 2017 Nov;309(9):709-719. doi: 10.1007/s00403-017-1772-x. Epub 2017 Aug 29.
Bullous pemphigoid (BP) is associated with higher mortality and coexisting comorbidities, some of them affecting poor prognosis. The aim of the study was to identify prognostic factors causing greater mortality both in the 1st and 3rd year of follow-up and to determine the 1-, 2-, 3-year mortality rates, standardized mortality ratio (SMR) in Polish BP patients. All patients with BP (a cohort of 205 patients, mean age 76.2 years) diagnosed between 5 January 2000 and 10 December 2013 in a referral unit for autoimmune bullous diseases at the university hospital in Poland were included retrospectively. Mortality data were obtained from the Centre for Document Personalization at the Minister of Interior and Administration. Our original observation was that prednisone in moderate dose (0.5 mg kg) in monotherapy was an independent risk factor of fatal prognosis in the 1st year of follow-up, assessed using multivariate analysis. We confirmed the strong correlation between neurological diseases and greater mortality. Both in the 1st and 3rd year of follow-up, dementia and Parkinson disease resulted in increased mortality. We also found that arrhythmias significantly increased mortality in the 1st and 3rd year of follow-up. The prognostic factors in BP changed over time of follow-up. In the 3rd year of observation, the age above 77, longer hospitalization and BP severity were associated with greater mortality. We observed poorer prognosis in BP patients than age-matched general Polish population. The 1-, 2-, 3-year mortality rates were 22.4, 31.2, 39.5% and SMR was 3.8 (95% CI 3.4-3.7).
大疱性类天疱疮（BP）与更高的死亡率和并存的合并症相关，其中一些合并症影响预后不良。本研究的目的是确定导致 1 年和 3 年随访时死亡率更高的预后因素，并确定波兰 BP 患者的 1 年、2 年、3 年死亡率、标准化死亡率比（SMR）。所有 205 例 BP 患者（平均年龄 76.2 岁）均于 2000 年 1 月 5 日至 2013 年 12 月 10 日在波兰大学医院的自身免疫性大疱性疾病转诊单位被回顾性纳入。死亡率数据从内政和行政部个人文件中心获得。我们的原始观察结果是，在多变量分析中，中等剂量（0.5mg/kg）泼尼松单药治疗是 1 年随访时致命预后的独立危险因素。我们证实了神经系统疾病与更高死亡率之间的强相关性。在 1 年和 3 年的随访中，痴呆症和帕金森病都导致死亡率增加。我们还发现心律失常在 1 年和 3 年的随访中显著增加了死亡率。BP 的预后因素随着随访时间的变化而变化。在 3 年的观察中，年龄超过 77 岁、住院时间延长和 BP 严重程度与更高的死亡率相关。我们观察到 BP 患者的预后比年龄匹配的普通波兰人群更差。1 年、2 年、3 年的死亡率分别为 22.4%、31.2%、39.5%，SMR 为 3.8（95%CI 3.4-3.7）。
