Eosinophilic fasciitis: clinical characteristics and response to methotrexate

Berianu, Florentina; Cohen, Marc D.; Abril, Andy; Ginsburg, William W.

doi:10.1111/1756-185x.12499

Cited by 41 publications

(59 citation statements)

References 13 publications

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“…Our cohort is similar to previously described series in many ways. The average age of onset, prolonged time from symptom onset to diagnosis, and frequency of eosinophilia align with all other previously published cohorts . The 1:1 female‐to‐male ratio seen here approximates the ratios reported by some, although others have reported a female predominance closer to 2:1 .…”

Section: Discussionsupporting

confidence: 90%

“…The average age of onset, prolonged time from symptom onset to diagnosis, and frequency of eosinophilia align with all other previously published cohorts . The 1:1 female‐to‐male ratio seen here approximates the ratios reported by some, although others have reported a female predominance closer to 2:1 . Inflammatory arthritis has been described as a clinical feature seen in EF, and was also seen in this cohort, although at a lower rate than in some previous reports .…”

Section: Discussionsupporting

confidence: 90%

“…Imaging modalities like ultrasound, positron emission tomography (PET) and MRI may provide early diagnosis and assessment of disease activity . Berianu et al reported a patient who had symptoms and signs affecting only the left side of the body, but MRI showed bilaterally symmetrical disease . This indicates that MRI may show changes before they are clinically apparent and that the disease can be more extensive than appreciated on clinical exam.…”

Section: Discussionmentioning

confidence: 99%

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Baseline characteristics and long‐term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years

et al. 2019

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Aim Eosinophilic fasciitis (EF) is a rare, fibrosing disorder of skin and subcutaneous tissue. This study was undertaken to describe its clinical and laboratory features and identify prognostic factors associated with outcome. Methods We conducted a retrospective review of all EF patients evaluated at our institution from 1 January1997 to 30 December 2016. Kaplan‐Meier methods were used to determine treatment response rates over time. Potential associations between baseline characteristics and complete response were examined using Cox models adjusted for age and sex. Time‐dependent covariates were used to examine treatment effects. Results We identified 89 EF patients, with a female‐to‐male ratio of 1:1. Clinical features included groove sign in 26 (29%), peau d'orange/dimpling in 22 (25%), inflammatory arthritis in 9 (10%) and muscle weakness in 9 (10%). Aldolase was elevated in 11/36 (31%). Complete response rate was 60% (95% confidence interval [CI]: 35‐75) at 3 years. Diagnostic delay was inversely associated with treatment response (hazards ratio: 0.84 per 1 month increase; 95% CI: 0.73‐0.98). No baseline characteristics correlated with treatment response, but a trend toward positive association of elevated aldolase, hypergammaglobulinemia and presence of hematologic disorders was noted. Methotrexate was the most commonly used immunosuppressant in 79%, hydroxychloroquine in 45%, mycophenolate mofetil in 18% and azathioprine in 8%. No single immunosuppressant agent was associated with a superior response during treatment. Conclusions EF is characterized by relatively high response rates. Consensus diagnostic criteria, standardized management algorithms, and large prospective multi‐center cohorts are needed to develop an evidence‐directed approach to this challenging condition.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 99%

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Baseline characteristics and long‐term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years

et al. 2019

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“…). In a report by Berianu et al ., eight out of 16 (50%) patients presented with this finding, and the disease duration of these patients was long. Furthermore, the groove sign is a depression along the course of the superficial veins, which is more pronounced on elevation of the affected limb.…”

Section: Guidelines For the Treatmentmentioning

confidence: 61%

Diagnostic criteria, severity classification and guidelines of eosinophilic fasciitis

Jinnin

Yamamoto

Asano

et al. 2017

The Journal of Dermatology

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We established diagnostic criteria and severity classification of eosinophilic fasciitis because there is no established diagnostic criteria or widely accepted severity classification of the disease. Also, there has been no clinical guideline for eosinophilic fasciitis, so we established its clinical guideline ahead of all over the world. In particular, the clinical guideline was established by clinical questions based on evidence-based medicine according to the New Minds Clinical Practice Guideline Creation Manual (version 1.0). We aimed to make the guideline easy to use and reliable based on the newest evidence, and to present guidance as specific as possible for various clinical problems in treatment of eosinophilic fasciitis.

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“…However, multiple recent retrospective studies have shown superior response rates in patients who are treated with a combination of HD SCS and an ISD, especially for weekly MTX (15–25 mg/week) [14, 16, 200]. These studies are retrospective and thus prone to confounding by indication.…”

Section: Treatmentmentioning

confidence: 99%

Morphea and Eosinophilic Fasciitis: An Update

et al. 2017

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Morphea, also known as localized scleroderma, encompasses a group of idiopathic sclerotic skin diseases. The spectrum ranges from relatively mild phenotypes, which generally cause few problems besides local discomfort and visible disfigurement, to subtypes with severe complications such as joint contractures and limb length discrepancies. Eosinophilic fasciitis (EF, Shulman syndrome) is often regarded as belonging to the severe end of the morphea spectrum. The exact driving mechanisms behind morphea and EF pathogenesis remain to be elucidated. However, extensive extracellular matrix formation and autoimmune dysfunction are thought to be key pathogenic processes. Likewise, these processes are considered essential in systemic sclerosis (SSc) pathogenesis. In addition, similarities in clinical presentation between morphea and SSc have led to many theories about their relatedness. Importantly, morphea may be differentiated from SSc based on absence of sclerodactyly, Raynaud’s phenomenon, and nailfold capillary changes. The diagnosis of morphea is often based on characteristic clinical findings. Histopathological evaluation of skin biopsies and laboratory tests are not necessary in the majority of morphea cases. However, full-thickness skin biopsies, containing fascia and muscle tissue, are required for the diagnosis of EF. Monitoring of disease activity and damage, especially of subcutaneous involvement, is one of the most challenging aspects of morphea care. Therefore, data harmonization is crucial for optimizing standard care and for comparability of study results. Recently, the localized scleroderma cutaneous assessment tool (LoSCAT) has been developed and validated for morphea. The LoSCAT is currently the most widely reported outcome measure for morphea. Care providers should take disease subtype, degree of activity, depth of involvement, and quality-of-life impairments into account when initiating treatment. In most patients with circumscribed superficial subtypes, treatment with topical therapies suffices. In more widespread disease, UVA1 phototherapy or systemic treatment with methotrexate (MTX), with or without a systemic corticosteroid combination, should be initiated. Disappointingly, few alternatives for MTX have been described and additional research is still needed to optimize treatment for these debilitating conditions. In this review, we present a state-of-the-art flow chart that guides care providers in the treatment of morphea and EF.

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Eosinophilic fasciitis: clinical characteristics and response to methotrexate

Abstract: We believe that MTX represents an effective treatment option for EF. The rarity of this disease would make a double-blind controlled trial study difficult to perform.

Cited by 41 publications

References 13 publications

Baseline characteristics and long‐term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years

Baseline characteristics and long‐term outcomes of eosinophilic fasciitis in 89 patients seen at a single center over 20 years

Diagnostic criteria, severity classification and guidelines of eosinophilic fasciitis

Morphea and Eosinophilic Fasciitis: An Update

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