Eosinophilic fasciitis

嗜酸性筋膜炎
  • 文章类型: Review
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  • 文章类型: Journal Article
    Morphea是一种自身免疫性纤维化皮肤病。嗜酸性筋膜炎(EF)被认为属于严重的硬皮病谱。我们进行了一项范围审查,评估了硬伤/EF患者的继发性癌症风险。副肿瘤性硬伤/EF和硬伤/EF继发于癌症治疗。搜索是使用MEDLINE进行的,Embase,Cochrane数据库,用于从开始到2022年9月发表的文章,遵循系统评价和范围评价荟萃分析的首选报告项目(PRISMA-ScR)指南,没有语言或日期限制。包括200项研究。其中,32项研究报道了硬伤/EF患者的继发性癌症,副肿瘤性硬伤/EF为45,癌症治疗诱导的硬伤/EF为125。虽然目前的证据仍然有限,数据表明,在硬伤患者中,继发性皮肤和胰腺恶性肿瘤的风险增加,特别是广义子类型。EF的数据不足。另一方面,副肿瘤性角膜是轶事,而一些观察性研究表明,约10%的EF病例可能是副肿瘤,主要在血液系统恶性肿瘤的背景下。放射疗法引起的硬伤很少见,在~0.2%的治疗患者中观察到,通常局限于治疗部位,除了预先存在自身免疫的患者。虽然报道了化疗引起的病例,免疫治疗的硬伤/EF病例正在出现,并且优先使用PD-1而不是CTLA-4抑制剂。这项研究受到所包括文章类型的限制(病例报告,病例系列和观察性研究),因此,需要对这一重要课题进行更多的研究。
    Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review assessing the risk of secondary cancer among morphea/EF patients, paraneoplastic morphea/EF and morphea/EF developing secondary to cancer therapy. The search was conducted using MEDLINE, Embase, Cochrane databases for articles published from inception to September 2022 following the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines with no language or date restrictions. Two hundred and one studies were included. Of these, 32 studies reported on secondary cancer in morphea/EF patients, 45 on paraneoplastic morphea/EF and 125 on cancer-treatment-induced morphea/EF. While the current evidence remains limited, data suggest an increased risk of secondary cutaneous and possibly pancreatic malignancy in morphea patients, particularly the generalized subtype. There were insufficient data for EF. On the other hand, paraneoplastic morphea was anecdotal, whereas several observational studies suggested that ~10% of EF cases may be paraneoplastic, primarily in the context of hematologic malignancies. Radiotherapy-induced morphea is rare, seen in ~0.2% of treated patients and is usually localized to the treatment site, except in patients with pre-existing autoimmunity. While chemotherapy-induced cases are reported, immunotherapy morphea/EF cases are emerging and are preferentially seen with PD-1 and not CTLA-4 inhibitors. This study is limited by the type of articles included (case reports, case series and observational studies), and hence, additional research on this important topic is needed.
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  • 文章类型: Journal Article
    由于与需要广泛鉴别诊断的其他自身免疫性疾病的常见症状,嗜酸性粒细胞性筋膜炎(EF)仍然是一种罕见的疾病,没有精确的诊断标准。本文以一名56岁男性患者为例,介绍了高分辨率肌肉骨骼超声检查和弹性成像在嗜酸性筋膜炎的诊断和随访中的应用。除了实验室数据,仪器数据,还有活检,在诊断过程和随访期间均使用肌肉骨骼超声检查(US),以客观评估患者病情变化和对治疗的反应.美国显示肌原纤维与浅筋膜相邻的组织紊乱,水肿,筋膜增厚和皮下水肿。此外,使用剪切波弹性成像(SWE)显示皮肤弹性显著降低.高频肌肉骨骼超声结合SWE是诊断EF和随访治疗后发生的变化的有效方法。基于它可以很容易地区分参与的底物,如皮肤,皮下组织,或者肌肉筋膜,超声可用于区分EF与其他皮肤和肌肉疾病。
    Eosinophilic fasciitis (EF) remains a rare condition without precise diagnostic criteria due to common symptoms with other autoimmune diseases requiring broad differential diagnosis. This paper describes the use of high-resolution musculoskeletal ultrasonography and elastography in the diagnosis and follow-up of eosinophilic fasciitis through the case of a 56-year-old male patient. In addition to laboratory data, instrumental data, and biopsy, musculoskeletal ultrasonography (US) was used both in the diagnostic process and in the follow-up period for an objective assessment of the changes in the patient\'s condition and response to treatment. The US showed disorganization of the myofibrils adjacent to the superficial fascia, edema, and thickening of the fascia and subcutaneous edema. In addition, the use of shear-wave elastography (SWE) demonstrated significantly reduced skin elasticity. High-frequency musculoskeletal ultrasound in combination with SWE is an effective method both for the diagnosis of EF and for the follow-up of the changes occurring after therapy. Based on the fact that it can easily differentiate the substrate of involvement, such as skin, subcutaneous tissue, or muscle fascia, ultrasound can be used to distinguish EF from other skin and muscle diseases.
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  • 文章类型: Journal Article
    嗜酸性筋膜炎(EF)是一种罕见的结缔组织疾病,其特征是亚急性发作的红斑,水肿,四肢和躯干的皮肤和软组织硬化。尽管假设有几个触发器与EF相关,嗜酸性筋膜炎(EF)的病因尚不清楚,已经提出了几种治疗方案来治疗这种疾病。在这篇文章中,我们报告了一个72岁的绅士有多种合并症,他出现在他的前臂弥漫性皮肤增厚的诊所,大腿,双腿两侧,在骨盆上。患者被诊断为EF,包括泼尼松在内的多种治疗方案均失败。甲氨蝶呤,利妥昔单抗,但最终有反应,并继续服用托珠单抗。在这篇文章中,我们将回顾当前对EF的理解,诊断方法,流行的治疗方法,并回顾使用托珠单抗的其他EF病例。
    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.
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  • 文章类型: Case Reports
    嗜酸性筋膜炎(EF),也被称为舒尔曼综合症,是一种罕见的硬皮病样疾病,其特征是硬结的急性发作,肿胀,红斑,皮肤和深筋膜的压痛,经常影响四肢。我们报告了一例51岁女性患者的嗜酸性筋膜炎,他们的EF诊断是根据临床评估和磁共振成像(MRI)的结果做出的,但没有皮肤活检。她接受了泼尼松龙和甲氨蝶呤的联合治疗,通过临床评估和MRI评估患者对治疗的反应.MRI可能是一种有用的非侵入性诊断工具,不仅支持而且在无法进行皮肤到肌肉活检或无法进行时确认EF的临床诊断。以及监测疾病活动和对治疗的反应。应进行进一步的前瞻性研究,以评估MRI诊断EF的精确敏感性和特异性,并创建更结构化的方案来指导EF的诊断和管理。
    Eosinophilic fasciitis (EF), also known as Shulman syndrome, is a rare scleroderma-like disorder that is characterized by an acute onset of induration, swelling, erythema, and tenderness of the skin and deep fascia, often affecting all four limbs. We report a case of eosinophilic fasciitis in a 51-year-old female patient, whose diagnosis of EF was made based on the findings from clinical evaluation and magnetic resonance imaging (MRI) but without skin biopsy. She was treated with a combination therapy of prednisolone and methotrexate, and her response to therapy was assessed via clinical assessment and MRI. MRI may be a useful non-invasive diagnostic tool for not only supporting but also confirming the clinical diagnosis of EF when a skin-to-muscle biopsy is not available or cannot be performed, as well as for monitoring disease activity and response to therapy. Further prospective studies should be conducted to evaluate the precise sensitivity and specificity of MRI in diagnosing EF and also to create more structured protocols to guide the diagnosis and management of EF.
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  • 文章类型: Case Reports
    胰高血糖素样肽单受体激动剂(GLP-1RA)是与人GLP-1的药理学组成和同源性不同的药物,并且最常用于治疗2型糖尿病和体重减轻。有与GLP-1RA相关的嗜酸性粒细胞不良反应的单独报告。我们介绍了一个42岁的女性患者,在开始每周皮下司马鲁肽后,在停用司马鲁肽和开始免疫抑制后,出现了嗜酸性筋膜炎,临床进展良好。提供了对先前报道的GLP-1RA的嗜酸性粒细胞不良事件的综述。
    Glucagon-like peptide one-receptor agonists (GLP-1 RA) are drugs that differ in their pharmacological composition and homology to human GLP-1 and are used most frequently for the treatment of type 2 diabetes and weight loss. There are isolated reports of eosinophilic adverse reactions associated with GLP-1 RA. We present the case of a 42-year-old female patient who, after starting weekly subcutaneous semaglutide, developed eosinophilic fasciitis with favorable clinical evolution after the discontinuation of semaglutide and the initiation of immunosuppression. A review of the eosinophilic adverse events that have been previously reported with GLP-1 RA is provided.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    背景:全身性硬皮和嗜酸性筋膜炎是难以治疗的炎症性和硬化性皮肤病。很少有报道说静脉注射免疫球蛋白是有益的,可能是由于它们的免疫调节和抗纤维化特性。
    目的:我们介绍了3例用静脉注射免疫球蛋白治疗的广泛性硬叶患者,并对文献进行了综述。
    方法:三名住院患者(两名男性,年龄66和65岁,分别,描述了一名67岁的女性)首次接受治疗的泛发性疣。
    结果:三名患者接受了静脉免疫球蛋白(每四周连续三到四天,1.5-2g/kg体重)治疗。所有患者都接受了皮质类固醇脉冲治疗,甲氨蝶呤治疗2例,霉酚酸酯治疗1例,分别。在所有患者中皮肤硬化的显著和稳定的改善是明显的,治疗开始后一到五个月。没有观察到不良事件。迄今为止,有12例报告,其中16例患者接受静脉注射免疫球蛋白治疗,患有广泛性硬叶或嗜酸性筋膜炎。该治疗在大多数患者中非常有效(9/16),并产生了良好的风险状况。
    结论:我们的病例增加了迄今为止有限的证据,即静脉内免疫球蛋白与糖皮质激素和常规免疫抑制剂联合给药是一种安全有效的治疗硬伤的方法。在未来的高质量研究中验证这些结果似乎是合适的。
    BACKGROUND: Generalized morphea and eosinophilic fasciitis are difficult-to-treat inflammatory and sclerosing skin diseases. Few cases have been reported in which intravenous immunoglobulins were of benefit, possibly owing to their immunomodulatory and antifibrotic properties.
    OBJECTIVE: We present three new patients with generalized morphea treated with intravenous immunoglobulins as well as a review of the literature.
    METHODS: Three hospitalized patients (two men, age 66 and 65 years, respectively, and a 67-year-old woman) with generalized morphea who received therapy for the first time are described.
    RESULTS: The three patients were treated with intravenous immunoglobulins (1.5-2 g/kg body weight over three to four consecutive days every four weeks). This was combined with corticosteroid pulse therapy in all patients, methotrexate in two patients and mycophenolate mofetil in one patient, respectively. Marked and steady improvement of skin sclerosis was evident in all patients, one to five months after treatment initiation. No adverse events were observed. To date, there are 12 reports of 16 patients with generalized morphea or eosinophilic fasciitis treated with intravenous immunoglobulins. The treatment was highly effective in the majority of patients (9/16) and yielded a favourable risk profile.
    CONCLUSIONS: Our cases add to the hitherto limited evidence that the administration of intravenous immunoglobulins in combination with glucocorticoids and conventional immunosuppressive agents is a safe and effective therapy against morphea. It seems appropriate to verify these results in future high-quality studies.
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