Department of Dermatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, Germany.
Department of Medical Informatics and Biostatistics, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania, Germany.
Indian J Dermatol Venereol Leprol. 2023 May-Jun;89(3):363-371. doi: 10.25259/IJDVL_705_2021.
Background Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. Aims The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. Methods We conducted a retrospective cohort study on newly diagnosed bullous pemphigoid patients between July 2001 and November 2019 in a referral unit for autoimmune blistering skin diseases in Romania. Results One hundred forty-eight patients were included in the study. The Kaplan-Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5-81.6%), 53.4% (45.7-62.2%), 43.6% (35.9-53%) and 31.3% (23.5-41.7%). The median follow-up among survivors was 48 months (interquartile range: 11-150). Ninety (60.8%) patients died during the follow-up period; of them, 38 (42.2%) had active disease at the time of death. Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Limitations This study lacks a control cohort to validate the obtained results. It was conducted in a retrospective manner in a single centre. In addition, indirect immunofluorescence microscopy was not performed in all patients. Conclusion Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients.
大疱性类天疱疮是最常见的皮下自身免疫性水疱病。到目前为止，大疱性类天疱疮的报告预后因素差异很大。目的：本研究旨在确定大疱性类天疱疮的总体生存率和预后因素。方法：我们对 2001 年 7 月至 2019 年 11 月在罗马尼亚自身免疫性水疱性皮肤病转诊单位新诊断的大疱性类天疱疮患者进行了回顾性队列研究。结果：本研究纳入 148 例患者。Kaplan-Meier 总体生存率在 1、3、5 和 10 年时分别为 74.2%（95%置信区间，67.5-81.6%）、53.4%（45.7-62.2%）、43.6%（35.9-53%）和 31.3%（23.5-41.7%）。幸存者的中位随访时间为 48 个月（四分位距：11-150）。90 例（60.8%）患者在随访期间死亡；其中 38 例（42.2%）死亡时疾病处于活动期。高龄、神经疾病、心脏瓣膜病、恶性肿瘤、他汀类药物使用、皮肤感染和广泛皮肤受累与预后较差相关，而局部皮质类固醇的使用与总体生存率增加相关。局限性：本研究缺乏对照组来验证获得的结果。它是在单一中心以回顾性方式进行的。此外，并非所有患者都进行间接免疫荧光显微镜检查。结论：除了年龄和神经合并症外，大疱性类天疱疮患者的预后还受到皮肤感染、心脏瓣膜病、他汀类药物使用和广泛皮肤受累的显著影响。局部皮质类固醇治疗与这些患者的生存增加相关。
