Treatment and Monitoring of Eosinophilic Fasciitis

Purpose of Review Eosinophilic fasciitis (EF) is a rare infammatory disease characterized by skin induration. Although some guidelines from scientifc societies exist, standard recommendations on monitoring and therapy are lacking. Recent Findings Current therapy for patients diagnosed with EF includ...

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Detalhes bibliográficos
Autores: Selva O'Callaghan, Albert|||0000-0003-2823-9761, Trallero Araguás, Ernesto|||0000-0003-3365-4521, Gil Vila, Albert|||0000-0003-2786-2009, Guillén-Del-Castillo, Alfredo|||0000-0003-0626-507X, Matas-Garcia, Ana|||0000-0002-9184-8079, Milisenda, José César|||0000-0003-0151-7872, Simeón-Aznar, Carmen Pilar|||0000-0003-3390-9029, Pinal Fernandez, Iago|||0000-0001-6338-9218
Formato: artículo
Fecha de publicación:2025
País:España
Recursos:Universitat Autònoma de Barcelona
Repositorio:Dipòsit Digital de Documents de la UAB
Idioma:inglés
OAI Identifier:oai:ddd.uab.cat:310297
Acesso em linha:https://ddd.uab.cat/record/310297
https://dx.doi.org/urn:doi:10.1007/s40674-024-00222-6
Access Level:acceso abierto
Palavra-chave:Eosinophilic fasciitis
Immunosuppressive treatment
Monitoring
Unmet needs
Groove sign
Descrição
Resumo:Purpose of Review Eosinophilic fasciitis (EF) is a rare infammatory disease characterized by skin induration. Although some guidelines from scientifc societies exist, standard recommendations on monitoring and therapy are lacking. Recent Findings Current therapy for patients diagnosed with EF includes glucocorticoids plus at least one immunosuppressive drug in cases of relapse or refractory disease. Methotrexate and mycophenolate mofetil are the most recommended, although recently a myriad of case reports or small series reporting the efectivity of biological agents or JAK inhibitors for treating relapses or refractory disease have been published. Anti-IL5 may have a role in those rare refractory cases with persistent eosinophilia. Intravenous immunoglobulins and photopheresis (in those centers with experience) may act as adjuvant therapies. Monitoring the disease activity is a cornerstone to ascertain if the treatment is useful or not. MRI, PET/TC, and more specifcally POCUS have recently demonstrated their value for assessing therapy response. Summary High-quality data focused on therapy and monitoring is lacking in EF. Strategies for improving scientifc quality of observational studies and consensus about "activity", "sequela", "relapse" or "refractoriness" terms in EF patients are necessary to implement prospective clinical trials and generate evidence-based medicine. Meanwhile we have to deal with the available information.