Clinical characteristics and outcomes of autoimmune blistering diseases in Japan.

Maria Rosa Noliza Encarnacion, Ryota Kawai, Hiroto Kuwabiraki, Nanaka Ban, Hisako Yoshida, Ayumi Shintani, Daisuke Tsuruta, Sho Hiroyasu
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Abstract

Autoimmune blistering diseases (AIBDs), including pemphigoid and pemphigus, are intractable dermatological disorders clinically characterized by blistering and erosion affecting mucosal membranes and the skin. Due to their rarity and the limited coverage for less severe cases under Japanese medical subsidies, comprehensive epidemiological analyses encompassing less severe cases have not been conducted in Japan. In this study, we analyzed the epidemiology of AIBDs in Japan, utilizing data from a Japanese nationwide database. We identified 9796 cases of bullous pemphigoid (BP), 62 cases of epidermolysis bullosa acquisita (EBA), 871 cases of pemphigus vulgaris (PV), and 578 cases of pemphigus foliaceous (PF). BP patients exhibited an older age distribution compared to EBA, PV, and PF, with median ages of 81, 72, 65, and 70 years, respectively. Higher rates of comorbidities such as Alzheimer's disease, spondylopathies, and extrapyramidal and movement disorders were observed only in BP cases, while other neurodegenerative disorders such as polyneuropathies, unspecified dementia, and schizophrenia were frequent in both BP and EBA. Dipeptidyl peptidase-4 inhibitors were more commonly prescribed before the onset of BP and EBA compared to PV and PF. Treatment patterns indicated that PV patients were more frequently administered higher doses of oral corticosteroids compared to other AIBDs. Additionally, aggressive therapies, including steroid pulse, intravenous immunoglobulin, and plasmapheresis therapies, were more frequently applied in PV cases. In-hospital mortality rates were higher in BP and EBA at 8.0% and 11.3%, respectively, compared to PV and PF at 2.8% and 5.9%, respectively. Kaplan-Meier analysis indicated that BP and EBA reached a 5-year in-hospital mortality rate of approximately 0.21 and 0.34, while PV and PF rates were approximately 0.07 and 0.11, respectively. The Cox hazard model revealed that higher age is the risk factor for in-hospital mortality in all diseases. Kaplan-Meier analysis indicated a cumulative steroid cessation probability of 0.25 at 3 years for BP, and at 6 and 5 years for PV and PF, respectively. The Cox hazard model revealed that higher age and lower maximum corticosteroid dose contribute to the steroid cessation probability in BP, PV, and PF. This study provides insights into the epidemiology, treatment patterns, comorbidities, and outcomes of AIBDs in Japan.

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日本自身免疫性水疱病的临床特征和治疗效果。
包括丘疹性荨麻疹和丘疹性荨麻疹在内的自身免疫性水疱病(AIBDs)是一种难治性皮肤病,临床特征是影响粘膜和皮肤的水疱和糜烂。由于其罕见性以及日本医疗补贴对较轻病例的覆盖范围有限,日本尚未对较轻病例进行全面的流行病学分析。在本研究中,我们利用日本全国数据库的数据分析了日本大疱性皮肤病的流行病学。我们发现了9796例大疱性类天疱疮(BP)患者、62例获得性表皮松解症(EBA)患者、871例寻常型天疱疮(PV)患者和578例叶状天疱疮(PF)患者。与EBA、PV和PF相比,BP患者的年龄分布较大,中位年龄分别为81岁、72岁、65岁和70岁。阿尔茨海默病、脊柱病、锥体外系疾病和运动障碍等合并症仅在 BP 病例中出现率较高,而多发性神经病、不明原因痴呆和精神分裂症等其他神经退行性疾病在 BP 和 EBA 中均很常见。与前列腺增生和前列腺肥大相比,二肽基肽酶-4 抑制剂在前列腺增生和前列腺肥大发病前更常见。治疗模式表明,与其他 AIBD 相比,PV 患者更常服用大剂量的口服皮质类固醇激素。此外,包括类固醇脉冲、静脉注射免疫球蛋白和血浆置换疗法在内的积极疗法也更多地应用于真性红斑狼疮病例。BP和EBA的院内死亡率分别为8.0%和11.3%,高于PV和PF的2.8%和5.9%。Kaplan-Meier 分析表明,BP 和 EBA 的 5 年院内死亡率分别约为 0.21 和 0.34,而 PV 和 PF 分别约为 0.07 和 0.11。Cox 危险模型显示,在所有疾病中,年龄越大,院内死亡率越高。Kaplan-Meier 分析表明,BP 在 3 年时停止使用类固醇的累积概率为 0.25,PV 和 PF 在 6 年和 5 年时停止使用类固醇的累积概率分别为 0.25。Cox 危险模型显示,年龄越大、皮质类固醇最大剂量越低,BP、PV 和 PF 停止使用类固醇的概率就越高。这项研究为了解日本AIBD的流行病学、治疗模式、合并症和预后提供了深入的见解。
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