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Diagnosis and Management of Pemphigus: recommendations by an International Panel of Experts
Journal of the American Academy of Dermatology ( IF 12.8 ) Pub Date : 2018-02-10
Dedee F. Murrell, Sandra Peña, Pascal Joly, Branka Marinovic, Takashi Hashimoto, Luis A. Diaz, Animesh A. Sinha, Aimee S. Payne, Maryam Daneshpazhooh, Rüdiger Eming, Marcel F. Jonkman, Daniel Mimouni, Luca Borradori, Soo-Chan Kim, Jun Yamagami, Julia S. Lehman, Marwah Adly Saleh, Donna A. Culton, Annette Czernik, John J. Zone, David Fivenson, Hideyuki Ujiie, Katarzyna Wozniak, Ayşe Akman-Karakaş, Philippe Bernard, Neil J. Korman, Frédéric Caux, Kossara Drenovska, Catherine Prost-Squarcioni, Snejina Vassileva, Ron J. Feldman, Adela Rambi Cardones, Johann Bauer, Dimitrios Ioannides, Hana Jedlickova, Francis Palisson, Aikaterini Patsatsi, Soner Uzun, Savas Yayli, Detlef Zillikens, Masayuki Amagai, Michael Hertl, Enno Schmidt, Valeria Aoki, Sergei A. Grando, Hiroshi Shimizu, Sharon Baum, Guiseppe Cianchini, Claudio Feliciani, Pilar Iranzo

Background

Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management,

Objective

We now present results from a subsequent Delphi consensus to broaden the generalizability of recommendations.

Methods

A preliminary survey, based on the European Dermatology Forum (EDF) and the European Academy of Dermatology and Venereology (EADV) guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology (AAD) conference. A second survey was sent following the meeting to more experts to achieve greater international consensus.

Results

The 39 experts participated in the first round of the Delphi-survey while 54 from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II based on Delphi results and meeting discussion.

Limitations

Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available.

Conclusions

We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first line therapy option for moderate to severe pemphigus.



中文翻译:

天疱疮的诊断和管理:国际专​​家小组的建议

背景

几个欧洲国家最近制定了国际天疱疮诊断和管理指南,这些指南有助于天疱疮管理的标准化,

客观的

现在,我们提出了随后的Delphi共识所得出的结果,以扩大建议的可推广性。

方法

根据欧洲皮肤病学论坛(EDF)和欧洲皮肤病与性病研究院(EADV)指南进行的初步调查已发送给国际专家小组,以确定共识程度。在2016年3月举行的年度美国皮肤病学会(AAD)会议上,国际大流行性疾病共识小组讨论了研究结果。会议之后,又向更多专家发送了第二份调查报告,以达成更大的国际共识。

结果

39名专家参加了第一轮德尔菲调查,来自21个国家的54名专家完成了第二轮调查。根据Delphi的结果和会议讨论,调查中的声明数量从Delphi I中的175个主题减少到Delphi II中的24个主题。

局限性

每个建议代表多数意见,因此可能无法反映所有可用的治疗选择。

结论

我们在这里提出了从德尔福过程中得到的建议。这一国际共识包括静脉CD20抑制剂作为中度至重度天疱疮的一线治疗选择。

更新日期:2018-02-10
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