Treatment Of Eosinophilic Fasciitis Research Articles

Tocilizumab in the Treatment of Eosinophilic Fasciitis: A Case Study and Review of Literature.

Eosinophilic fasciitis (EF) is a rare connective-tissue disorder that is characterised by subacute onset of erythema, oedema, and induration of the skin and soft tissues of the limbs and trunk. Although several triggers have been hypothesised to be associated with EF, the aetiology of eosinophilic fasciitis (EF) is still unclear, and several treatment regimens have been proposed to treat this disease. In this article, we report a case of a 72-year-old gentleman with multiple comorbidities who presented to the clinic for diffuse skin thickening present on his forearms, thighs, legs bilaterally, and over the pelvis. The patient was diagnosed with EF and failed multiple treatment regimens including prednisone, methotrexate, rituximab, but finally responded and was maintained on tocilizumab. In this article, we will review the current understanding of EF, diagnostic approach, popular treatments and review other cases of EF in which tocilizumab was used.

Scleroderma and Related Disorders

Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility. This review contains 11 highly rendered figures, 3 tables, and 72 references.

Eosinophilic Fasciitis: an Updated Review on Diagnosis and Treatment.

First recognized in 1974, eosinophilic fasciitis (EF) is a fibrosing disorder of the fascia with characteristic cutaneous and hematologic manifestations. This review discusses recent trends in the diagnosis and treatment of EF. Although fascial biopsy has classically been considered the gold standard for making a diagnosis of EF, radiologic imaging, particularly magnetic resonance imaging, has been increasingly used for both diagnosis and monitoring of treatment response. Systemic corticosteroids remain the first-line treatment for EF; however, their often prolonged use in the treatment of EF has prompted a search for adjunctive therapies. Methotrexate has emerged as the leading corticosteroid-sparing agent for EF. Since EF was initially described over 40years ago, important diagnostic and therapeutic progress has been made. Future efforts should be directed at the pursuit of prospective studies including clinical trials and evidence-based guidelines.

Scleroderma and Related Disorders

Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility. This review contains 11 highly rendered figures, 3 tables, and 72 references.

Scleroderma and Related Disorders

Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility. This review contains 11 highly rendered figures, 3 tables, and 72 references.

Efficacy of Tocilizumab in the treatment of Eosinophilic fasciitis: Report of one case

A 43-year-old man was diagnosed with an Eosinophilic fasciitis with cutaneous and articular involvement. The patient experienced an early response with high-dose corticosteroids achieving a global remission of disease. Nevertheless, during the steroids tapering phase, he presented a new flare and subsequently developed a corticosteroid refractory disease. The addition of Methotrexate in monotherapy then associated with an anti-tumor necrosis factor agent did not show any additional benefit. Therefore Tocilizumab, a humanized monoclonal antibody against the interleukin-6 receptor, was initiated achieving an immediate response that persists after 36months of follow-up. The use of this biological agent allows prednisone withdrawal at 3months and remission of both articular and cutaneous manifestations at 6months. This report describes for the first time the efficacy of an anti interleukin-6 agent in Eosinophilic fasciitis treatment.

A57: Four Prolonged Cases of Eosinophilic Fasciitis in Children

Background/Purpose:Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by a progressive inflammatory thickening of skin and soft tissues. Diagnosis is often based on characteristic clinical and biopsy findings, and treatment remains challenging. Pediatric data describing clinical course and treatment recommendations remain scarce.Methods:This is a case series of all patients diagnosed with EF at our institution in the last 8 years. A retrospective chart review was conducted and data collected on clinical signs and symptoms and laboratory data at diagnosis, disease course, and treatment outcomes. Descriptive statistics were used when appropriate.Results:We describe four pediatric patients with EF, with a mean follow‐up of 54 months. Three of the four patients had preceding diagnoses before beginning treatment for EF. All patients had peripheral eosinophilia at diagnosis, two had positive anti‐thyroid antibodies, and two had high levels of total cholesterol. Treatment induction in all patients consisted of intravenous methylprednisone (30mg/kg/day to a maximum of 1g daily for 3 days). All patients required at least one steroid‐sparing agent (methotrexate at 15mg/m2/dose and/or mycophenolate mofetil (MMF) at 600mg/m2/dose) for disease maintenance post‐induction. The mean length of treatment with methotrexate was 23 months, discontinued in only one patient. The mean length of treatment with MMF was 8.3 months. All patients had improvement in skin findings, and all abnormal laboratory findings normalized after treatment with intravenous corticosteroid. One patient had a disease flare less than 12 months after being weaned off cortico‐steroids and methotrexate. Another patient developed a second autoimmune disease five years after treatment for EF. All patients had residual skin changes, and 3 patients had signs of lipodystrophy and/or subcutaneous fat atrophy after treatment.Conclusion:EF is often difficult to diagnose and has a variable course in children. We report four cases of prolonged EF, the only four EF patients diagnosed in our practice. Our data suggest a more severe course at earlier age of onset, with higher likelihood of recurrence. Our data also confirm the efficacy of corticosteroid treatment as first‐line therapy, and suggest a role for methotrexate as a promising steroid‐sparing agent. This is the first case series to describe successful treatment of EF with the addition of mycophenolate mofetil to a treatment course of methotrexate and corticosteroid therapy. Three of our four patients required triple therapy for disease control, which may be suggestive of a more severe disease course in the pediatric population.

FRI0401 Eosinophilic fasciitis (EF) clinical characteristics and response to mtx therapy

BackgroundThe treatment of EF is still a challenge for rheumatologists.ObjectivesTo report our experience with fourteen EF patients on MTX followed over a period of 1 to 14 years in the...

D-penicillamine in the treatment of eosinophilic fasciitis: case reports and review of the literature

Eosinophilic fasciitis (EF) is a rare disease characterized by symmetrical thickness and hardening of the skin, especially localized to forearms and thorax, with eosinophilia. Corticosteroids represent the first-line therapy, even if some patients are scarcely responsive and/or may develop important side effects due to long-term treatment. Here, we describe three cases of EF, two of them refractory to previous steroid therapy, successfully treated with D-penicillamine. The present clinical observations together with the updated review of the literature suggest usefulness of D-penicillamine in EF patients, as well as its potential steroid-sparing value.

A Case of Steroid and Methotrexate-Resistant Eosinophilic Fasciitis Treated with Adalimumab

Eosinophilic fasciitis (EF) is a rare fibrosing disorder characterized by painful swelling and induration of the limbs and trunk, characteristic histology with sclerosis and lymphocytic inflammation affecting the fascia. The cause and pathogenesis of EF are still unknown and current therapies include glucocorticoids with or without use of immunosuppressive agents. Recently, there have been several case reports documenting the efficacy of a TNF alpha inhibitor in EF following a steroid-resistant disease course. However, there has been no report on the experience in treatment of EF with a TNF alpha inhibitor in Korea. Hence, we report a case of steroid and methotrexate-resistant EF which was successfully treated with adalimumab, along with a review of the relevant articles.

Extracorporeal photochemotherapy in the treatment of eosinophilic fasciitis.

Eosinophilic fasciitis (EF) is a rare connective tissue disorder characterized clinically by symmetrical swelling, induration and thickening of the skin and histologically by thickening of the fascia with chronic inflammatory infiltrate containing eosinophils. The disease is classified in the spectrum morphea/systemic sclerosis and treated with systemic steroids and other immunosuppressant drugs. The purpose of this study was to use extracorporeal photochemotherapy (ECP) in patients with EF to evaluate the effectiveness of this therapy. Three patients affected by EF were treated with ECP because they failed to respond or with contraindications to immunosuppressant treatment. The patients underwent ECP with a UVAR XTS apparatus. Subjects were treated on two consecutive days at 2-week intervals for the first 3 months and thereafter every 4 weeks on the basis of clinical response. The patients were assessed before therapy and then monthly by means of a clinical score. Changes in affected areas were evaluated at predetermined points by computerized skin elastometry (Cutometer SEM 474). After 1 year of therapy we found considerable improvement of clinical parameters in two cases. There was less striking improvement in the other case. These clinical results were confirmed by the elastometry measurements. All patients reported improved quality of life, which enabled a reduction in the dose of immunosuppressants. ECP emerged as a safe and effective therapy in association with low doses of immunosuppressants in our three patients. A randomized comparative multicentre study between ECP as single therapy and ECP plus immunosuppressants and conventional therapies is required to firmly establish photopheresis as a possible basic treatment to combine with conventional therapies for EF.