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Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature

Identifieur interne : 000535 ( Istex/Corpus ); précédent : 000534; suivant : 000536

Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature

Auteurs : Lindsay Bischoff ; Chris T. Derk

Source :

RBID : ISTEX:927BE1E0C5F83A8591229BF73B72B0C2E6A9071B

English descriptors

Abstract

Background  Eosinophilic fasciitis is a rare scleroderma‐like illness. The clinical spectrum of the disease has evolved since its initial description. Methods  We identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded. Results  Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 ± 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 ± 6.1 months before diagnosis. The mean initial absolute peripheral blood eosinophil count was 1188 ± 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine. At a mean follow up of 17.6 months (range 2–94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment. Conclusion  High dose corticosteroid treatment lasting longer than a month with or without an immunosuppressive agent helped most patients with eosinophilic fasciitis, best results seen in those patients who were initiated treatment early on after their first symptoms.

Url:
DOI: 10.1111/j.1365-4632.2007.03544.x

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ISTEX:927BE1E0C5F83A8591229BF73B72B0C2E6A9071B

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Eosinophilic fasciitis is a rare scleroderma‐like illness. The clinical spectrum of the disease has evolved since its initial description.</p>
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We identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded.</p>
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Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 ± 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 ± 6.1 months before diagnosis. The mean initial absolute peripheral blood eosinophil count was 1188 ± 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine. At a mean follow up of 17.6 months (range 2–94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment.</p>
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<correspondenceTo> 
Chris T. Derk,
<sc>md, ms</sc>
Assistant Professor of Medicine Division of Rheumatology Thomas Jefferson University 613 Curtis Bldg 1015 Walnut Street Philadelphia PA 19107 USA
E‐mail:
<email>Chris.Derk@jefferson.edu</email>
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<title type="main">Eosinophilic fasciitis: demographics, disease pattern and response to treatment: report of 12 cases and review of the literature</title>
<title type="shortAuthors">
<i>Bischoff and Derk</i>
</title>
<title type="short">Eosinophilic fasciitis</title>
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<personName>
<givenNames>Lindsay</givenNames>
<familyName>Bischoff</familyName>
<degrees>Senior Medical Student</degrees>
</personName>
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<givenNames>Chris T.</givenNames>
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<degrees>Asst Prof</degrees>
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<unparsedAffiliation>From the Jefferson Medical College, Philadelphia PA, and Division of Rheumatology, Thomas Jefferson University, Philadelphia, PA 19107, USA</unparsedAffiliation>
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<title type="main">Abstract</title>
<p>
<b>Background </b>
Eosinophilic fasciitis is a rare scleroderma‐like illness. The clinical spectrum of the disease has evolved since its initial description.</p>
<p>
<b>Methods </b>
We identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded.</p>
<p>
<b>Results </b>
Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 ± 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 ± 6.1 months before diagnosis. The mean initial absolute peripheral blood eosinophil count was 1188 ± 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine. At a mean follow up of 17.6 months (range 2–94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment.</p>
<p>
<b>Conclusion </b>
High dose corticosteroid treatment lasting longer than a month with or without an immunosuppressive agent helped most patients with eosinophilic fasciitis, best results seen in those patients who were initiated treatment early on after their first symptoms.</p>
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<title>Eosinophilic fasciitis</title>
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<abstract lang="en">Background  Eosinophilic fasciitis is a rare scleroderma‐like illness. The clinical spectrum of the disease has evolved since its initial description. Methods  We identified all patients diagnosed with eosinophilic fasciitis over the past 10 years at our scleroderma clinic. Demographics, disease pattern, serologies, tissue pathology and reponse to treatment were all recorded. Results  Twelve patients with eosinophilic fasciitis were identified in our clinic over the past 10 years. The mean age at diagnosis was 49.8 ± 9.8 years, with nine female and three male patients. The first symptoms were noticed at an average of 8.8 ± 6.1 months before diagnosis. The mean initial absolute peripheral blood eosinophil count was 1188 ± 1059 cells/L. Two patients had a monoclonal gammopathy, and two had positive ANA titers. All patients received corticosteroids, 10 of whom received the equivalent dose of > 20 mg/day of prednisone for more than a month. Five patients received hydroxychloroquine, two received methotrexate, one received cyclosporine, one received topical tacrolimus, and one received sulfasalazine. At a mean follow up of 17.6 months (range 2–94 months), 8 patients had a good response to treatment, 2 patients had no effect, and 2 patients had a poor response to treatment. Conclusion  High dose corticosteroid treatment lasting longer than a month with or without an immunosuppressive agent helped most patients with eosinophilic fasciitis, best results seen in those patients who were initiated treatment early on after their first symptoms.</abstract>
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