Open Orphanet-ES opened 3 months ago
MCecile-US14
commented
3 months ago Dear @Orphanet-ES,
Thank you for submitting and transfering this request. Please note that due to the Orphanet Nomenclature pack release 2024, a high number of prioritized requests this will be evaluate after the summer break
Thank you for your understanding Kind Regards, Marie-Cécile
moi-epiUS14
commented
1 month ago Thank you for raising the concern.
Indeed, EoE was removed from our base a few years ago as it is no longer rare as the result of revision in diagnostic criteria in 2017/1028.
When this decision was taken, EoE and PPI were still considered to be different entities, therefore, PPI remained in our base.
However, if PPI is no longer considered to be an independent entity from EoE, but rather a part of it, then consequently, PPI should be removed from our base as well.
Orphanet-ES
commented
4 weeks ago Dear @moi-epiUS14 ,
Thanks for your answer.
Please, let us know when the disease is removed from Orphanet so we can inform the expert.
Kind regards,
María Elena
Dear Marie-Cécile,
Following with the issue related to the EoE consultation you received and forwarded to us, we come back to you to suggest that it is revised by the nomenclature team if you esteemed it necessary.
As we mentioned in our last email to Elisa Gallego, it looked like it remained controversial whether a difference should be made between PPI responsive and unresponsive patients.
However, Elisa has contacted a gastroenterologist specialized in the disease and according to his opinion, backed up by some published work, the classification which differentiates between PPI responsive and unresponsive patients is not in use anymore and current guidelines are aimed at EoE in general.
Please find here some of the publications they have shared:
-AGA Institute and the Joint Task Force on Allergy-Immunology Practice Parameters Clinical Guidelines for the Management of Eosinophilic Esophagitis. https://doi.org/10.1053/j.gastro.2020.02.038 “It is important to note that a European and an International consensus recommendation have recently removed the PPI trial from the diagnostic criteria of EoE.7,8 After the exclusion of secondary causes of esophageal eosinophilia, symptomatic esophageal eosinophilia is now viewed as synonymous with EoE. PPIs are positioned as an effective, primary therapeutic option for certain patients with EoE. Based on their longstanding safety profile and ease of administration, patients may prefer to start with this form of therapy before trials of glucocorticosteroids or elimination diets. It should be emphasized that direct comparison of the efficacy of PPI and other medical or dietary EoE therapies is limited because, up to this time, most trials in EoE have excluded patients with esophageal eosinophilia that responded to a PPI (formerly denoted as PPIresponsive esophageal eosinophilia)”
-Updated International Consensus Diagnostic Criteria for Eosinophilic Esophagitis: Proceedings of the AGREE Conference. DOI: 10.1053/j.gastro.2018.07.009 “Conclusion: A tremendous amount of progress has been made in the understanding of EoE in the last 2 decades spanning clinical presentation, epidemiology, genetics, pathogenesis, treatment, and outcomes. With such a rapid evolution of knowledge, diagnostic criteria must also evolve. Although EoE and GERD were first believed to be distinct and separable by a PPI trial, there was increasing recognition that the relationship was far more complex, that they could co-exist, and that each might influence the other. With the identification of patients who responded to PPI treatment, it was not initially known if PPI-REE was a subtype of EoE, an atypical manifestation of GERD, or a unique entity. Now, the evidence suggests that in many cases PPI-REE is indistinguishable from EoE and ta PPIs are better classified as a treatment for esophageal eosinophilia that may be due to EoE than as a diagnostic criterion. These updated international consensus criteria reflect this concept. As the field continues to develop and the research questions identified during this process are answered, the criteria presented here will evolve in the context of new data and advances.”
''After this study, subsequent guidelines excluded esophageal pH monitoring as a criterion for the diagnosis of EoE, but continued to consider the response to PPI as a sufficient reason to rule out EoE”. [6]. Hence, in 2011, “PPI-responsive esophageal eosinophilia” was defined to classify patients with apparent EoE who achieved complete remission after treatment with PPIs, replacing GERD as the main differential diagnosis of EoE. From then until today, numerous studies have shown that patients who respond to PPIs share not only the findings mentioned above but also at the genetic and molecular level 7; Therefore, the concept of "PPI-responsive esophageal eosinophilia" is currently out of use and is part of the EoE spectrum and should not be considered a separate entity and therefore treatment with PPIs is a therapeutic option and not a diagnostic criterion”.
In the light of this, perhaps it should be discussed whether the entity “Proton-pump inhibitor-responsive esophageal eosinophilia” should remain an entity within the Orphanet classification.
We look forward to your reply.
Thank you in advance and kind regards,
Virginia Corrochano James