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Erdheim-Chester disease (ECD) is an histiocytosis of the L-group, characterised by multiorgan infiltration of histiocytes that often carry oncogenic mutations.1 In the past few years, several studies have progressively and carefully mapped ECD genome, and several mutations have been described, most of them activating the mitogen-activated protein kinases (MAPK) pathway.1 These discoveries led to new therapies targeting BRAF or MEK in patients with ECD.2 Despite these findings, ECD pathogenesis still remains undetermined. Patients with ECD present a systemic inflammatory disease, with not only macrophages but also several other immune cells infiltrating affected tissues. A few studies have tried to explore the immune phenotype of ECD, with cytokine dosage in plasma and/or immunohistochemistry analysis, suggesting a complex network of inflammatory cytokines and chemokines.3 In order to explore the inflammatory phenotype of ECD, we realised immune phenotyping of circulating immune cells of 13 patients with ECD (details on patients’ characteristics are available in online supplementary table 1) and …
Handling editor Josef S Smolen
Contributors Study conception and design: MP, AC and TTM. Acquisition of data and experiments performance: MP, AC and BR. Analysis of data, drafting and writing of the manuscript: MP, AC, JH and TTM. All authors contributed to reviewing the manuscript and approved the final version for publication.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.