SCLERODERMA

硬皮病
  • 文章类型: Journal Article
    系统性硬化症(SSc),作为一种以免疫失调和血管病变为特征的自身免疫性风湿性疾病,影响多个器官。由于其症状对医疗保健系统的负担很高,本研究旨在探讨益生菌补充剂对SSc患者的影响.
    我们在PubMed中使用预定义的搜索词搜索了电子数据库,Scopus,和ISIWebofScience截至2023年6月。该研究包括评估益生菌补充剂对患有SSc的成年患者的影响的随机对照试验。纳入研究的结果报告为加权平均差(WMD),置信区间为95%(CI)。
    4项研究符合纳入标准,纳入荟萃分析。共有176例SSc患者。结果表明,益生菌补充剂对含有反流的胃肠道(GI)症状具有显着影响(WMD:-0.36,95%CI:-0.51至-0.22,p值<0.001),气体和腹胀(WMD:-0.88,95%CI:-1.05至-0.7,p值<0.001)。然而,便秘的结果(WMD:-0.12,95%CI:-0.27至0.04,p值=0.13),腹泻(WMD:-0.14,95%CI:-0.31至0.03,p值=0.10),大便失禁(WMD:0.04,95%CI:-0.06至0.15,p值=0.43)不显著。
    补充益生菌可以缓解SSc中的一些胃肠道并发症。然而,由于研究数量有限,需要更多精心设计的研究来加强这些结果。
    UNASSIGNED: Systemic sclerosis (SSc), as an autoimmune rheumatic disease characterized by immune dysregulation and vasculopathy, affects multiple organs. Due to the high burden of its symptoms on the health care system, this study aims to investigate the effects of probiotic supplements in patients with SSc.
    UNASSIGNED: We searched electronic databases with predefined search terms in PubMed, Scopus, and ISI Web of Science up to June 2023. Randomized controlled trials that evaluated the effects of probiotic supplementation in adult patients suffering from SSc were included in the study. Results of the included studies were reported as weighted mean difference (WMD) with a 95 % confidence interval (CI).
    UNASSIGNED: Four studies met the inclusion criteria and were included in the meta-analysis. There was a total of 176 SSc patients. The results show a significant effect of probiotics supplementation on gastrointestinal (GI) symptoms containing reflux (WMD: -0.36, 95 % CI: -0.51 to -0.22, p-value <0.001), gas and bloating (WMD: -0.88, 95 % CI: -1.05 to -0.7, p-value<0.001). However, the results for constipation (WMD: -0.12, 95 % CI: -0.27 to 0.04, p-value = 0.13), diarrhea (WMD: -0.14, 95 % CI: -0.31 to 0.03, p-value = 0.10), and fecal incontinence (WMD: 0.04, 95 % CI: -0.06 to 0.15, p-value = 0.43) were insignificant.
    UNASSIGNED: Supplementing with probiotics may alleviate a few numbers of GI complications in SSc. Nevertheless, due to the limited number of studies, more well-designed studies are needed to strengthen these results.
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  • 文章类型: Journal Article
    背景:目前对局部硬皮病(LS)的治疗已被证明可以阻止疾病活动,但是对这些治疗的患者体验知之甚少,对于未来临床试验的最佳测量策略也没有达成共识.
    目的:对结果和措施类型的文献进行范围审查(即临床医生-,患者-,和护理人员报告的)用于已发表的LS治疗研究。
    方法:在在线数据库中搜索与评估LS治疗效果相关的文章,特别关注儿科。
    结果:在168项研究中,最常用的结局是通过临床医生报告的评估测量的皮肤疾病活动度和损伤.最常引用的测量是局部硬皮病皮肤评估工具(LoSCAT)。很少使用患者报告的结果测量(PROM)。
    结论:一些研究只是模糊地报告了所使用的措施,并且该审查产生的临床试验数量很少。
    结论:除了用临床医生报告的措施评估疾病活动外,该领域可以通过包括症状和功能的高质量PROM来获得关于患者体验的关键知识。需要使用各种结果和措施进行更多的临床试验,以确定最适合LS患者的治疗过程。
    BACKGROUND: Current treatment for localized scleroderma (LS) has been shown to halt disease activity, but little is still known about patient experiences with these treatments, nor is there consensus about optimal measurement strategies for future clinical trials.
    OBJECTIVE: Conduct a scoping review of the literature for the types of outcomes and measures (i.e. clinician-, patient-, and caregiver-reported) utilized in published treatment studies of LS.
    METHODS: Online databases were searched for articles related to the evaluation of treatment efficacy in LS with a special focus on pediatrics.
    RESULTS: Of the 168 studies, the most common outcomes used were cutaneous disease activity and damage measured via clinician-reported assessments. The most frequently cited measure was the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT). Few patient-reported outcome measures (PROMs) were used.
    CONCLUSIONS: Some studies only vaguely reported the measures utilized, and the review yielded a low number of clinical trials.
    CONCLUSIONS: In addition to evaluating disease activity with clinician-reported measures, the field could obtain critical knowledge on the patient experience by including high-quality PROMs of symptoms and functioning. More clinical trials using a variety of outcomes and measures are necessary to determine the most suitable course of treatment for LS patients.
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  • 文章类型: Journal Article
    腕管综合征(CTS)是世界上最常见的压迫性神经病。几个条件可能有助于CTS的发展,比如肥胖,重复的手腕运动,怀孕,遗传易感性和类风湿性关节炎(RA)炎症。CTS的特点是广泛的病理生理因素,包括增加的压力,机械性创伤和对穿过腕部隧道的正中神经的缺血性损伤。在目前的叙事文献综述中,研究了风湿性疾病(RD)与CTS发生的关系。流行病学,临床,检查了关系的临床和发病机制方面。CTS是RA中最常见的神经系统表现,和RA的发病率,银屑病关节炎与CTS密切相关。CTS与系统性红斑狼疮,干燥综合征,白塞病和系统性硬化症较弱。在这些情况下,CTS的患病率与普通人群相似.随着CTS发生率的增加,了解共同机制并做出早期诊断需要限制疼痛和费用。当RD患者出现腕关节疼痛等症状时,手指刺痛或麻木,应该怀疑CTS。这种怀疑不应该用RD来解释。为了准确评估RD患者,评估过程中应包括详细的电生理检查。诊断算法应包括对RD患者的神经肌肉超声或磁共振成像。
    Carpal tunnel syndrome (CTS) is the most commonly occurring type of entrapment neuropathy in the world. Several conditions may contribute to the development of CTS, such as obesity, repetitive wrist movements, pregnancy, genetic predisposition and rheumatoid arthritis (RA) inflammation. CTS is characterized by a wide range of pathophysiological factors, including increased pressure, mechanical trauma and ischemic damage to the median nerve that runs through the wrist tunnel. In the present narrative literature review, the way rheumatic diseases (RDs) contribute to CTS occurrence is investigated. The epidemiological, clinical, paraclinical and pathogenesis aspects of the relationship are examined. CTS is the most common neurological finding in RA, and incidences of RA, psoriatic arthritis and CTS are closely related. The association of CTS with systemic lupus erythematosus, Sjögren\'s syndrome, Behcet\'s disease and systemic sclerosis is weaker. In these cases, the prevalence of CTS is similar to that in the general population. As the occurrence of CTS is increasing, understanding the common mechanism and making an early diagnosis are required to limit pain and costs. When patients with RD present with symptoms such as wrist pain, tingling sensations or numbness in their fingers, CTS should be suspected. This suspicion should not be interpreted in terms of RD. To accurately evaluate patients with RD, a detailed electrophysiological examination should be included in the evaluation process. A diagnostic algorithm should include neuromuscular ultrasound or magnetic resonance imaging for patients with RD.
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  • 文章类型: Journal Article
    准分子光是NB-UVB的一种亚型,发射308nm波长,并能提供有针对性的光疗治疗。皮肤细胞对308nm光的吸收导致各种常见和紫外线反应性皮肤病的治疗反应,比如牛皮癣和白癜风,和耐光皮肤病,如结节性痒疹,局限性硬皮病,生殖器苔藓硬化,和环状肉芽肿,皮肤T细胞淋巴瘤,在其他人中。准分子光不良反应少,患者总体耐受性好,此外,它可以在难以访问的地方进行。本文旨在阐述准分子光在当前皮肤病学中的治疗基础和应用。
    Excimer light is a subtype of NB-UVB that emits a 308 nm wavelength, and can provide targeted phototherapy treatment. The absorption of 308 nm light by skin cells leads to therapeutic response in various common and ultraviolet-responsive skin diseases, such as psoriasis and vitiligo, and photo-resistant skin diseases such as prurigo nodularis, localized scleroderma, genital lichen sclerosis, and granuloma annulare, cutaneous T-cell lymphomas, among others. Excimer light has few adverse reactions and overall is well tolerated by patients, furthermore, it can be performed in places that are difficult to access. This article aims to explain the therapeutic bases and applications of excimer light in current dermatology.
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  • 文章类型: Journal Article
    硬皮病的口腔和牙齿表现极为常见,然而,它们在风湿病学中经常被忽视,在牙科中却知之甚少。先前的研究表明,有必要了解硬皮病患者及其护理参与者的口腔和牙科经历。这项范围审查的目的是,第一次,全面绘制关于硬皮病口腔和牙齿表现的识别和管理的已知信息,硬皮病患者是如何经历这些的,并探索硬皮病良好口腔和牙齿护理的障碍和促成因素的关键特征。使用六个数据库(Embase,PubMed,心理信息,ASSIA,Scopus和SSCI),根据系统审查和荟萃分析的首选报告项目-范围审查的扩展。灰色文献也包括在内。如果全文和摘要以英文提供,则研究有资格纳入。2002年至2022年出版,重点关注成人硬皮病口腔和牙科护理的概念,无论是关于识别和管理,最佳实践的推动者和障碍,或者病人的经历和幸福。旨在了解患者生活经历的定性研究在文献中存在显着差距。同样,在风湿病中,对硬皮病的口腔和牙齿表现缺乏关注。确定了三个关键特征,这将促进研究和临床实践中的最佳实践:多学科护理的必要性;集中患者体验的必要性;以及减轻牙科护理障碍的必要性。我们得出的结论是,牙科领域对硬皮病的认识有所提高,并简化了牙科和风湿病学科之间的转诊程序,为了能够早期识别和管理硬皮病,是至关重要的。
    Oral and dental manifestations of scleroderma are extremely common, yet they are often overlooked within rheumatology and poorly understood within dentistry. Previous research has indicated the need to understand the oral and dental experiences of people living with scleroderma and those involved in their care. This scoping review aims, for the first time, to comprehensively map what is known regarding the identification and management of oral and dental manifestations of scleroderma, how these are experienced by people living with scleroderma, and to explore key characteristics of barriers and enablers to good oral and dental care in scleroderma. A scoping review was conducted using six databases (Embase, PubMed, PsychINFO, ASSIA, Scopus and SSCI), according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses - extension for Scoping Review. Grey literature was also included. Studies were eligible for inclusion if the full text and abstract were available in English, published between 2002 and 2022, and focused on the concept of oral and dental care in adults with scleroderma, either relating to identification and management, enablers and barriers to best practice, or patient experiences and well-being. Qualitative research which seeks to understand patients\' lived experiences was a notable gap in the literature. Similarly, there was a significant lack of focus on the oral and dental manifestations of scleroderma in rheumatology. Three key features were identified which would facilitate best practice in research and clinical contexts: the necessity of multidisciplinary care; the necessity of centralising patient experience; and the necessity of mitigating barriers to dental care. We conclude that increased awareness of scleroderma within dentistry and streamlining referral procedures between the disciplines of dentistry and rheumatology, to enable the early identification and management of scleroderma, are crucial.
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  • 文章类型: Letter
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  • 文章类型: Systematic Review
    目的:对系统性硬化症(SSc)患者进行放疗的担忧源于SSc表现和放疗毒性的潜在恶化。我们进行了系统评价,以评估放疗对SSc结局和放疗相关毒性的影响。
    方法:MEDLINE,Embase,搜索Cochrane系统评价数据库和Cochrane对照试验中央登记册以进行SSc和放射治疗。纳入标准为SSc诊断,随后的癌症发展,和放射治疗。结果是SSc表现(皮肤增厚,肺纤维化,和SSc耀斑)和放疗毒性(急性和晚期),利用不良事件通用术语标准进行分级。1级和2级毒性被归类为非严重和3-5级为严重。
    结果:121例接受放疗的SSc患者(平均年龄56.4岁,83.3%为女性,中位放疗剂量50Gy),大多数患者未出现放疗后SSc皮肤增厚恶化(92.7%)或肺部并发症(90.3%).在回顾性研究中,非严重急性不良反应的平均发生率为57.3%,严重不良反应的平均发生率为25.8%,而非严重的晚期不良反应发生率为32.4%,严重的为24%。
    结论:尽管大多数SSc患者放疗后SSc表现没有明显恶化,急性和晚期毒性的风险是可变的。这些发现表明,虽然放疗可能是SSc癌症患者的可行选择,这需要谨慎。
    OBJECTIVE: Concerns regarding offering radiotherapy to patients with systemic sclerosis (SSc) stem from the potential worsening of SSc manifestations and radiotherapy toxicity. We conducted a systematic review to evaluate the effects of radiotherapy on SSc outcomes and radiotherapy-related toxicity.
    METHODS: MEDLINE, Embase, Cochrane Database of Systematic Reviews, and Cochrane Central Register of Controlled Trials were searched for SSc and radiotherapy. Inclusion criteria were SSc diagnosis, subsequent cancer development, and radiotherapy exposure. Outcomes were SSc manifestations (cutaneous thickening, pulmonary fibrosis, and SSc flare) and radiotherapy toxicity (acute and late) using Common Terminology Criteria for Adverse Events for grading. Grade 1 and 2 toxicities were categorized as nonsevere and grade 3 to 5 toxicities as severe.
    RESULTS: Of 121 patients with SSc undergoing radiotherapy (mean age 56.4 years, 83.3% female, median radiotherapy dose 50 Gy), most did not show worsened SSc skin thickening (74.5%) or pulmonary complications (74%) post radiotherapy. In retrospective studies, the average rates of acute adverse effects were 57.3% for nonsevere and 25.8% for severe, whereas the rates of late adverse effects were 32.4% for nonsevere and 24% for severe.
    CONCLUSIONS: Although most patients with SSc do not exhibit significant worsening of SSc manifestations post radiotherapy, there is a variable risk of acute and late toxicity. These findings suggest that although radiotherapy may be a viable option for patients with cancer with SSc, it requires caution.
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  • 文章类型: Systematic Review
    近年来,研究方法的快速发展使单细胞分析成为可能。系统性硬化症(SSc),一种以免疫异常为特征的疾病,纤维化,血管病变,也是各种分析的主题。总结迄今为止积累的SSc单细胞分析结果,加深对SSc的理解。2023年6月23日,使用了四个数据库进行数据库搜索。建议评估的评估等级,根据PRISMA指南制定和评估证据的确定性。该分析于2023年7月完成。纳入了358名SSc患者的17项研究。三项研究使用PBMC,六种用过的皮肤,9例患有SSc间质性肺病(ILD)的二手肺,和一个使用肺SSc肺动脉高压(PAH)。研究的细胞包括免疫细胞,如T细胞,自然杀伤细胞,单核细胞,巨噬细胞,和树突状细胞,以及内皮细胞,成纤维细胞,角质形成细胞,肺泡I型细胞,基底上皮细胞,平滑肌细胞,间皮细胞,等。本系统综述揭示了单细胞分析的结果,表明PBMC,皮肤,SSc-ILD,和SSc-PAH显示与免疫异常相关的细胞的激活和功能障碍,纤维化,血管病变,分别。
    In recent years, rapid advances in research methods have made single cell analysis possible. Systemic sclerosis (SSc), a disease characterized by the triad of immune abnormalities, fibrosis, and vasculopathy, has also been the subject of various analyses. To summarize the results of single cell analysis in SSc accumulated to date and to deepen our understanding of SSc. Four databases were used to perform a database search on 23rd June 2023. Assessed Grading of Recommendations Assessment, Development and Evaluation certainty of evidence were performed according to PRISMA guidelines. The analysis was completed on July 2023. 17 studies with 358 SSc patients were included. Three studies used PBMCs, six used skin, nine used lung with SSc-interstitial lung diseases (ILDs), and one used lung with SSc-pulmonary arterial hypertension (PAH). The cells studied included immune cells such as T cells, natural killer cells, monocytes, macrophages, and dendritic cells, as well as endothelial cells, fibroblasts, keratinocytes, alveolar type I cells, basal epithelial cells, smooth muscle cells, mesothelial cells, etc. This systematic review revealed the results of single cell analysis, suggesting that PBMCs, skin, SSc-ILD, and SSc-PAH show activation and dysfunction of cells associated with immune-abnormalities, fibrosis, and vasculopathy, respectively.
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  • 文章类型: Journal Article
    系统性硬化症(SSc)是一种慢性自身免疫性结缔组织疾病,影响全球超过200万人。它通过血管病变表现出来,异常的免疫反应,和纤维化导致多个器官功能障碍。该疾病分为两种亚型:局限性皮肤SSc和弥漫性皮肤SSc。硬皮病可以影响呼吸道的重要器官,心脏,肾,眼,和皮肤病并发症。该疾病的眼部表现可发生在眼睛的前部和后部。与疾病相关的眼前段变化包括眼睑皮肤重塑,干眼症,结膜异常.该疾病对眼后段的影响主要导致视网膜微循环系统的病变和视神经的异常。这篇综述提供了与硬皮病相关的眼部并发症的详细见解。
    Systemic sclerosis (SSc) is a chronic autoimmune connective tissue disease that affects more than 2 million people worldwide. It manifests through vasculopathy, an abnormal immunological response, and fibrosis leading to dysfunction of the multiple organs. The disease is categorized into two subtypes: limited cutaneous SSc and diffuse cutaneous SSc. Scleroderma can affect vital organs with respiratory, cardiac, renal, ocular, and dermatological complications. The ocular manifestations of the disease can occur in the anterior and posterior segments of the eye. Changes in the anterior segment related to the disease include eyelid skin remodeling, dry eye syndrome, and conjunctival abnormalities. The disease\'s impact on the posterior segment of the eye mostly causes pathologies in the retinal microcirculatory system and abnormalities in the optic nerve. This review provides detailed insights into ocular complications associated with scleroderma.
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  • 文章类型: Meta-Analysis
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