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一项关于天疱疮和类天疱疮治疗方法的10年观察性研究。

A 10-Year Observational Study on Treatment Approaches in Pemphigus and Pemphigoid.

作者信息

Pereira Cristiana Palmela, Santos Rui, Ferreira Leonor, Martins Matilde, Rodrigues Ana, Augusto Diana, Filipe Paulo

机构信息

Faculty of Dental Medicine, University of Lisbon.

Faculty of Medicine, University of Lisbon, Portugal.

出版信息

Acta Stomatol Croat. 2025 Jun;59(2):190-198. doi: 10.15644/asc59/2/8.

DOI:10.15644/asc59/2/8
PMID:40641586
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12239650/
Abstract

OBJECTIVES

The aim of this study was to evaluate epidemiological data on pemphigus vulgaris and pemphigoid, with special focus on characteristics, clinical manifestations, and treatment approaches.

MATERIALS AND METHODS

This study includes patients who have been diagnosed with pemphigus vulgaris or pemphigoid at the Dermatology Service of the Santa Maria Local Health Unit between the years 2013 and 2022. The statistical software IBM SPSS® version 29, with Clopper-Pearson 95% confidence intervals (CI) was used for proportions.

RESULTS

The total number of patients included in this study is 99 patients with pemphigus vulgaris (49 females, 50 males) and 227 with pemphigoid patients (108 females, 119 males). Patients who have been treated for pemphigus vulgaris, the most prescribed medicine were corticosteroids, followed by the second most prescribed, drugs which were immunosuppressants. Additionally rituximab was used in 21 patients. The interval of the disease was 304 days; with an interval of no symptom disease was 415 days for relapsed patients. Similarly, for pemphigoid, the primary treatment used was with corticosteroids, which were also used for the main treatment, in many cases it was combined with other medicines, such as: antihistamines, antibiotics, and immunosuppressants. The average cure time was 148 days, with 32.0% of patients claiming they experienced relapse.

CONCLUSION

To conclude, we observed that corticosteroids, particularly prednisolone, remain still the most important pharmacological treatment for pemphigus vulgaris and pemphigoid. However, rituximab is an effective pharmacological alternative for pemphigus, while the use of clobetasol propionate is increasing and becoming as one of the preferred treatments for pemphigoid.

摘要

目的

本研究旨在评估寻常型天疱疮和类天疱疮的流行病学数据,特别关注其特征、临床表现和治疗方法。

材料与方法

本研究纳入了2013年至2022年间在圣玛丽亚地方卫生单位皮肤科被诊断为寻常型天疱疮或类天疱疮的患者。使用IBM SPSS® 29统计软件及Clopper-Pearson 95%置信区间(CI)计算比例。

结果

本研究纳入的患者总数为99例寻常型天疱疮患者(49例女性,50例男性)和227例类天疱疮患者(108例女性,119例男性)。接受寻常型天疱疮治疗的患者中,最常开具的药物是皮质类固醇,其次是第二常开具的免疫抑制剂。此外,21例患者使用了利妥昔单抗。病程为304天;复发患者无症状病程为415天。同样,对于类天疱疮,主要治疗药物是皮质类固醇,在许多情况下还与其他药物联合使用,如抗组胺药、抗生素和免疫抑制剂。平均治愈时间为148天,32.0%的患者称经历过复发。

结论

总之,我们观察到皮质类固醇,尤其是泼尼松龙,仍然是寻常型天疱疮和类天疱疮最重要的药物治疗。然而,利妥昔单抗是天疱疮的一种有效药物替代方案,而丙酸氯倍他索的使用正在增加并成为类天疱疮的首选治疗方法之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/b9eb9fbddcc8/ASC_59-2_190-198-f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/8a669b47f206/ASC_59-2_190-198-f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/2469a943bede/ASC_59-2_190-198-f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/cd2eca0ee835/ASC_59-2_190-198-f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/b9eb9fbddcc8/ASC_59-2_190-198-f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/8a669b47f206/ASC_59-2_190-198-f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/2469a943bede/ASC_59-2_190-198-f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/cd2eca0ee835/ASC_59-2_190-198-f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9432/12239650/b9eb9fbddcc8/ASC_59-2_190-198-f4.jpg

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本文引用的文献

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Biomarkers in Pemphigus Vulgaris: A Systematic Review.寻常型天疱疮的生物标志物:系统评价。
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A Comprehensive Review on the Efficacy of Anti-CD20 Therapies in Pemphigus Treatment.抗CD20疗法在天疱疮治疗中疗效的综合综述
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Pemphigus and pemphigoids: Clinical presentation, diagnosis and therapy.天疱疮和类天疱疮:临床表现、诊断与治疗
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J Eur Acad Dermatol Venereol. 2023 Jun;37(6):1118-1134. doi: 10.1111/jdv.18931. Epub 2023 Mar 25.
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Rituximab, Omalizumab, and Dupilumab Treatment Outcomes in Bullous Pemphigoid: A Systematic Review.利妥昔单抗、奥马珠单抗和度普利尤单抗治疗大疱性类天疱疮的疗效:系统评价。
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Long-term oral prednisolone exposure in primary care for bullous pemphigoid: population-based study.长期口服泼尼松龙治疗大疱性类天疱疮:基于人群的研究。
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Targeting the FcRn: A Novel Approach to the Treatment of Pemphigus.靶向 FcRn:天疱疮治疗的新方法。
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