eISSN: 2084-9834
ISSN: 0034-6233
Reumatologia/Rheumatology
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2/2019
vol. 57
 
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Artykuł oryginalny

Eosinophilic fasciitis (Shulman’s disease): review and comparative evaluation of seven patients

Joana Urzal
,
Miriam Cimbron
,
Teresa Mendonça
,
Fátima Farinha

Data publikacji online: 2019/04/29
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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.





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