Clinical course and outcomes of pemphigus vulgaris and foliaceus: A retrospective study using a nationwide database in Japan
Clinical course and outcomes of pemphigus vulgaris and foliaceus: A retrospective study using a nationwide database in Japan
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DOI：
10.1111/1346-8138.16641
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发表时间：
2022-11
期刊：
影响因子：
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通讯作者：
H. Miyachi;T. Konishi;Yohei Hashimoto;H. Matsui;K. Fushimi;T. Inozume;H. Yasunaga
中科院分区：
文献类型：
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作者：
H. Miyachi;T. Konishi;Yohei Hashimoto;H. Matsui;K. Fushimi;T. Inozume;H. Yasunaga
天疱疮是一种危及生命的自身免疫性水疱疾病。由于罕见，患者特征、疗程和结果尚不清楚。为了描述天疱疮的背景、治疗和结局，我们从日本全国住院患者数据库中确定了2598例寻常型天疱疮患者和1186例叶状天疱疮患者。寻常型天疱疮患者较年轻（62岁vs 72岁，P < 0.001），合并症较少，并且比叶状天疱疮患者更有可能入住大容量医院（38% vs 30%, P < 0.001）。与叶状天疱疮患者相比，寻常型天疱疮患者接受了更积极的治疗，包括类固醇脉冲治疗、静脉注射免疫球蛋白或血浆置换（48% vs 42%, P = 0.001）；具体来说，在年龄<70岁的患者中，寻常型天疱疮组比叶状天疱疮组更有可能接受积极治疗（52% vs 45%），而在年龄≥70岁的患者中没有显著差异（40% vs 40%）。与叶状天疱疮患者相比，寻常型天疱疮患者更频繁地使用免疫抑制剂（30%对26%，P = 0.015）和镇痛药，包括阿片类药物（45%对36%，P < 0.001），而局部皮质类固醇的使用频率较低（32%对48%，P < 0.001）。寻常型天疱疮患者的住院死亡率低于叶状天疱疮患者（2.2% vs 4.0%, P = 0.002）；在按年龄分层的比较中，两组的死亡率相当（<70岁的患者死亡率为0.6%，≥70岁的患者死亡率为6.1%）。总体而言，寻常型天疱疮患者比叶状天疱疮患者住院时间长10天，住院费用高。我们的研究结果为了解日本天疱疮管理的当前趋势提供了有用的信息。
Pemphigus is a life‐threatening autoimmune blistering disease. Patient characteristics, treatment courses, and outcomes remain unclear owing to its rarity. To describe the background, treatment, and outcomes of pemphigus, we identified 2598 patients with pemphigus vulgaris and 1186 patients with pemphigus foliaceus from a nationwide inpatient database in Japan. Patients with pemphigus vulgaris were younger (62 vs 72 years, P < 0.001), had fewer comorbidities, and were more likely to be admitted to high‐volume hospitals (38% vs 30%, P < 0.001) than those with pemphigus foliaceus. Patients with pemphigus vulgaris had undergone more aggressive treatment, including steroid pulse therapy, intravenous immunoglobulin, or plasmapheresis, compared with those with pemphigus foliaceus (48% vs 42%, P = 0.001); specifically, in patients aged <70 years, the pemphigus vulgaris group was more likely to undergo aggressive treatment than the pemphigus foliaceus group (52% vs 45%), whereas there was no significant difference in patients aged ≥70 years (40% vs 40%). Immunosuppressive agents (30% vs 26%, P = 0.015) and analgesics, including opioids (45% vs 36%, P < 0.001), were used more frequently, whereas topical corticosteroids were used less frequently (32% vs 48%, P < 0.001) in patients with pemphigus vulgaris compared with those with pemphigus foliaceus. In‐hospital mortality was lower in patients with pemphigus vulgaris than in those with pemphigus foliaceus (2.2% vs 4.0%, P = 0.002); in the comparison stratified by age, the mortality was equivalent among the two groups (0.6% in patients aged <70 years and 6.1% in those aged ≥70 years). Overall, patients with pemphigus vulgaris had a 10‐day longer hospitalization period and higher hospitalization costs than those with pemphigus foliaceus. Our findings provide useful information for understanding the current trends in the management of pemphigus in Japan.