Aziz Syed N, Proano Lucia, Cruz Claudio, Tenemaza Maria Gabriela, Monteros Gustavo, Hassen Gashaw, Baskar Aakash, Argudo Jennifer M, Duenas Jonathan B, Fabara Stephanie P
Internal Medicine, Shaheed Suhrawardy Medical College, Dhaka, BGD.
Internal Medicine, Pontificia Universidad Católica del Ecuador, Quito, ECU.
Cureus. 2022 Jun 14;14(6):e25935. doi: 10.7759/cureus.25935. eCollection 2022 Jun.
Erdheim Chester disease (ECD) is a type of histiocytosis characterized by a variable clinical presentation. The treatment of ECD is complex and mainly unknown. We aim to conduct a literature review of the treatment of ECD and consolidate the knowledge about the most recent and updated treatment for ECD. To conduct the systematic review, we used the preferred reporting items for systematic reviews and meta-analysis (PRISMA) protocol. To analyze the bias, we used the Cochrane collaboration risk-of-bias tool to assess the bias. We included observational studies and clinical trials on humans, which were written in English. Papers not fulfilling the objective of our study were excluded. Overall, the drug showed efficacy in the clinical trials, showing prolonged improvement and high rates of response rate. Overall, the drug was not well tolerated, and patients had a long list of side effects. Nevertheless, the drug seems to be a good option for second-line treatment for patients with ECD and BRAFV600 mutation.
Erdheim-Chester病（ECD）是一种组织细胞增多症，其临床表现多样。ECD的治疗复杂且主要未知。我们旨在对ECD的治疗进行文献综述，并巩固有关ECD最新和更新治疗方法的知识。为进行系统评价，我们使用了系统评价和荟萃分析的首选报告项目（PRISMA）方案。为分析偏倚，我们使用Cochrane协作偏倚风险工具来评估偏倚。我们纳入了以英文撰写的关于人类的观察性研究和临床试验。不符合我们研究目的的论文被排除。总体而言，该药物在临床试验中显示出疗效，表现为改善时间延长和高缓解率。总体而言，该药物耐受性不佳，患者有一长串副作用。然而，对于患有ECD和BRAFV600突变的患者，该药物似乎是二线治疗的一个不错选择。
