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Eosinophilic fasciitis (Shulman's disease): review and comparative evaluation of seven patients
Joana Urzal1, Miriam Cimbron2, Teresa Mendonça3
1Department of Internal Medicine, Hospital Professor Doutor Fernando Fonseca, Portugal.
Objectives:
Eosinophilic fasciitis (EF) was described in 1974 by Shulman as a rare fibrosing connective tissue disease of unknown etiology. An undetermined trigger is thought to lead to the degranulation of eosinophils that interact with fibroblasts and express fibrogenic cytokines including the transforming factor of tumor growth a and b and interleukins 1 and 6. The purpose of this study was to summarize seven cases of EF in a central hospital.
Material And Methods:
This was a retrospective and descriptive study of a population with EF of a central hospital. All patients diagnosed with EF in a hospital unit were admitted to the study between January 1, 2005, and April 30, 2018.
Results:
A total of seven patients diagnosed with EF were analyzed. The median age of the population at the time of diagnosis was 56 years, and 57% of the patients were women. All patients had elevated peripheral eosinophilia and sedimentation rate, and only one patient had hypergammaglobulinemia. All patients had edema and cutaneous thickening of the limbs, 57% had constitutional symptoms, and 57% had inflammatory arthritis with joint contracture. Prednisolone (PDN) therapy was initiated in all patients, and only in two was the association of PDN with methotrexate (MTX) initially performed. In one patient triple therapy of PDN, MTX, and cyclosporine was required. At the time of this publication, only one patient maintains active disease, and tocilizumab has been initiated.
Conclusions:
Recent studies show a more favorable response from the combination of PDN and MTX than from PDN alone. Considering the rarity of the disease, more long-term studies are needed regarding the etiopathogenetics, progression, recurrence of EF, and new effective therapies.
Insights
Eosinophilic fasciitis (EF) is a rare fibrosing disease. This study of seven cases suggests combination therapy with Prednisolone and Methotrexate may be more effective than Prednisolone alone for treating EF.
Area of Science:
- Rheumatology
- Dermatology
- Immunology
Background:
- Eosinophilic fasciitis (EF) is a rare fibrosing connective tissue disease of unknown cause.
- It involves eosinophil degranulation, fibroblast interaction, and fibrogenic cytokine release.
- Potential triggers are unknown, but cytokines like TGF-β and ILs are implicated.
Purpose of the Study:
- To summarize seven cases of Eosinophilic fasciitis (EF) treated at a central hospital.
- To analyze the clinical presentation, treatment, and outcomes of EF patients.
- To contribute to understanding this rare fibrosing condition.
Main Methods:
- Retrospective and descriptive study design.
- Inclusion of all EF patients diagnosed between January 1, 2005, and April 30, 2018.
- Analysis of demographic, clinical, laboratory, and therapeutic data.
Main Results:
- Seven EF patients analyzed; median age 56, 57% female.
- All presented with elevated eosinophilia, ESR, limb edema, and skin thickening.
- Prednisolone (PDN) was initiated in all; PDN + Methotrexate (MTX) in two, and triple therapy in one. One patient remains active and initiated tocilizumab.
Conclusions:
- Combination therapy of PDN and MTX shows promise over PDN alone for EF.
- Further long-term research is needed on EF etiopathogenesis, progression, recurrence, and novel therapies.
- Tocilizumab is a potential new therapeutic option for refractory EF.
