Treatment and Monitoring of Eosinophilic Fasciitis

Abstract

Eosinophilic fasciitis (EF) is a rare inflammatory disease characterized by skin induration. Although some guidelines from scientific societies exist, standard recommendations on monitoring and therapy are lacking. 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 effectivity 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 specifically POCUS have recently demonstrated their value for assessing therapy response. High-quality data focused on therapy and monitoring is lacking in EF. Strategies for improving scientific 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.