Systemic scleroderma

  • 文章类型: Journal Article
    本研究旨在分析系统性硬皮病(SSc)患者与正常皮肤相比的真皮厚度变化,并比较弥漫性和局限性皮肤受累的临床形式。研究组由诊断为SSc且病史不超过5年的女性患者组成。超声检查的感兴趣区域包括第三根手指的近端指骨,第二掌骨间隙,和前臂下三分之一的延伸表面。该研究包括20名诊断为SSc的患者和14名对照。根据临床形式将SSc患者细分为两个亚组。与对照组相比,SSc患者在所有三个皮肤区域的平均测量值均较高,在手和前臂区域具有统计学上的显着差异。表现为弥漫性SSc的患者,平均而言,与所有检查的皮肤区域的有限SSc相比,皮肤厚度更高,仅在前臂区域有统计学上的显着差异。根据疾病表现,仅在弥漫性SSc组中肺动脉高压的存在方面观察到显著差异.总之,皮肤超声是诊断和量化系统性硬皮病皮肤纤维化的一种有用和可利用的成像方法。
    This study aims to analyze the changes in dermal thickness in patients with systemic scleroderma (SSc) in comparison with normal skin and also compare clinical forms with diffuse and limited cutaneous involvement. The study group consisted of female patients diagnosed with SSc with a disease history not exceeding 5 years. The areas of interest for ultrasound examination included the proximal phalanx of the third finger, the second intermetacarpal space, and the extension surface of the lower third of the forearm. The study included 20 patients diagnosed with SSc and 14 controls. SSc patients were subdivided into two subgroups based on the clinical form. Compared to the control group, patients with SSc had higher mean measurements in all three skin areas, with statistically significant differences in the hand and forearm areas. Patients with diffuse SSc displayed, on average, higher skin thickness compared to limited SSc in all skin areas examined, with a statistically significant difference only in the forearm area. Based on disease manifestations, significant differences were observed only with regard to the presence of pulmonary hypertension in the diffuse SSc group. In conclusion, skin ultrasound is a useful and accessible imaging method for diagnosing and quantifying dermal fibrosis in systemic scleroderma.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    免疫炎性风湿性疾病(IRD)的发病机制是以慢性炎症为基础的,其关键机制之一可能是巨噬细胞的异常激活,导致免疫系统的进一步破坏。
    目的:。这项研究的目的是评估IRD患者循环单核细胞的促炎激活。
    方法:。该研究涉及149名参与者(53名类风湿关节炎(RA)患者,45例系统性红斑狼疮(SLE),34例系统性硬皮病(SSc),和17名没有IRD的参与者)30至65岁。在通过免疫磁性分离从血液中获得的单核细胞的原代培养物中研究了基底和脂多糖(LPS)刺激的单核细胞分泌。细胞因子肿瘤坏死因子α(TNF-α)的定量评估,白细胞介素1β(IL-1β),以及通过ELISA在培养液中进行趋化因子单核细胞趋化蛋白-1(MCP-1)。单核细胞的促炎激活被计算为LPS刺激的和基础分泌物的比率。
    结果:。结果表明,在所有IRD患者组中,所有研究的细胞因子的基础分泌均显着增加,SLE组除分泌IL-1β外,与对照相比。与对照组相比,IRD患者LPS刺激的TNF-α分泌增加,MCP-1减少;仅SSc组的LPS刺激的IL-1β分泌与对照组明显不同。在RA组中,与对照组相比,所有细胞因子的单核细胞活化均降低;在SLE组中,对于TNF-α和MCP-1;在SSc组中,对于MCP-1。
    结论:。IRD患者单核细胞促炎激活的减少是由于细胞因子的基础分泌水平高,这可能导致这些疾病中足够的免疫反应的破坏,并且是慢性炎症发病机理中的重要环节。
    The pathogenesis of immunoinflammatory rheumatic diseases (IRDs) is based on chronic inflammation, one of the key mechanisms of which may be abnormal activation of macrophages, leading to further disruption of the immune system.
    OBJECTIVE: . The objective of this study was to evaluate the proinflammatory activation of circulating monocytes in patients with IRDs.
    METHODS: . The study involved 149 participants (53 patients with rheumatoid arthritis (RA), 45 patients with systemic lupus erythematosus (SLE), 34 patients with systemic scleroderma (SSc), and 17 participants without IRDs) 30 to 65 years old. Basal and lipopolysaccharide (LPS)-stimulated secretion of monocytes was studied in a primary culture of monocytes obtained from blood by immunomagnetic separation. Quantitative assessment of the cytokines tumor necrosis factor α (TNF-α), interleukin 1β (IL-1β), as well as the chemokine monocyte chemoattractant protein-1 (MCP-1) was carried out in the culture fluid by ELISA. Proinflammatory activation of monocytes was calculated as the ratio of LPS-stimulated and basal secretions.
    RESULTS: . It was shown that the basal secretion of all studied cytokines was significantly increased in all groups of patients with IRDs, except for the secretion of IL-1β in the SLE group, compared to the control. LPS-stimulated secretion of TNF-α was increased and MCP-1 was decreased in patients with IRDs compared to the control group; LPS-stimulated IL-1β secretion only in the SSc group significantly differed from the control group. In the RA group, monocyte activation was reduced for all cytokines compared to the control; in the SLE group, for TNF-α and MCP-1; in the SSc group, for MCP-1.
    CONCLUSIONS: . The decrease in proinflammatory activation of monocytes in patients with IRDs is due to a high level of basal secretion of cytokines, which can lead to disruption of the adequate immune response in these diseases and is an important link in the pathogenesis of chronic inflammation.
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  • 文章类型: Letter
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  • 文章类型: Journal Article
    抑郁症是世界范围内日益严重的问题。它们的特征还在于高合并症,包括来自皮肤病的圈子。自身免疫性疾病似乎与抑郁共病特别相关,提出了他们可能的共同病理机制的问题。搜索了PubMed数据库,关注2016年后公布的结果。抑郁症与牛皮癣的特定相互关系,特应性皮炎,斑秃,脓疱病,发现狼疮和系统性硬皮病。上述疾病并存的一种可能的解释是,炎症理论可能适用于抑郁症,其中的各种元素也适用于自身免疫性疾病。
    Depressive disorders are a growing problem worldwide. They are also characterized by high comorbidity, including from the circle of dermatological diseases. Autoimmune diseases seem to be particularly correlated with depressive comorbidity, raising the question of their possible common pathomechanism. The PubMed database was searched, focusing on results published after 2016. A particular reciprocal correlation of depressive disorders with psoriasis, atopic dermatitis, alopecia areata, impetigo, lupus and systemic scleroderma was found. One possible explanation for the co-occurrence of the above diseases is that the inflammatory theory may be applicable to depression, the various elements of which also apply to autoimmune diseases.
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  • 文章类型: Case Reports
    背景:使用免疫检查点抑制剂的免疫治疗并非没有包括风湿病在内的免疫相关不良事件(irAEs)。
    方法:我们报告了一例罕见的病例,一名47岁的女性转移性黑色素瘤患者在开始使用nivolumab后发展为系统性硬皮病。病人表现出炎性关节痛,早晨僵硬,和该疾病的经典皮肤表现。临床评估还显示腕管综合征,心脏受累,和呼吸困难。RNA聚合酶III抗体呈阳性。Nivolumab,抗PD-1抗体,被认为是这种情况的潜在触发因素。
    结论:据我们所知,这是在符合疾病分类标准的文献中描述的黑色素瘤中,纳武单抗诱导的系统性硬皮病首例.该病例强调了接受免疫检查点抑制剂的患者需要提高对免疫相关不良事件的认识。强调及时干预和进一步研究。
    BACKGROUND: Immunotherapy using immune checkpoint inhibitors is not devoid of immune-related adverse events (irAEs) including rheumatological conditions.
    METHODS: We report a rare case of a 47-year-old woman with metastatic melanoma who developed systemic scleroderma after initiating nivolumab. The patient displayed inflammatory arthralgias, morning stiffness, and classical cutaneous manifestations of the disease. Clinical evaluations also revealed carpal tunnel syndrome, cardiac involvement, and dyspnea. RNA-Polymerase III antibodies were positive. Nivolumab, an anti-PD-1 antibody, was considered as a potential trigger for this condition.
    CONCLUSIONS: To our knowledge, this is the first case of nivolumab-induced systemic scleroderma in the context of melanoma described in the literature that fulfills the classification criteria of the disease. This case underscores the need for increased awareness of immune-related adverse events in patients receiving immune checkpoint inhibitors, emphasizing timely intervention and further research.
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  • 文章类型: Journal Article
    (1)背景:本研究旨在评估系统性硬化症患者的牙周炎负担及其可能的关联。以及一些潜在的危险因素和两种潜在的诊断生物标志物可能导致这种关联的程度。(2)方法:这项横断面研究包括测试组(系统性硬化症患者)和对照组(非系统性硬化症患者)。两组都受益于医疗,牙周检查和唾液取样,以确定唾液流速和两个炎症生物标志物(钙卫蛋白,psoriasin)。建立系统性硬化症严重程度量表。(3)结果:在研究组中,相当的牙周炎发生率为88.68%和85.85%,分别,已确定。不同程度的系统性硬化症患者牙周炎的严重程度差异无统计学意义。或抗着丝粒和抗SCL70抗体阳性。肌肉骨骼病变在III/IV期牙周炎中(n=33,86.84%)明显高于I/II期(n=1,100%,n=3,37.5%,分别)(p=0.007)。两组显示出相当水平的炎症介质。在弥漫性和局限性形式的系统性硬化症中,钙卫蛋白和银屑病素水平没有显着差异。(4)结论:在当前研究的局限性内,系统性硬化症和牙周炎之间没有关联,或者在他们的危险因素之间,可以证明。
    (1) Background: This study aimed to assess the periodontitis burden in systemic sclerosis patients and the possible association between them, and the degree to which some potential risk factors and two potential diagnostic biomarkers may account for this association. (2) Methods: This cross-sectional study included a test group (systemic sclerosis patients) and a control group (non-systemic sclerosis patients). Both groups benefited from medical, periodontal examination and saliva sampling to determine the salivary flow rate and two inflammatory biomarkers (calprotectin, psoriasin). A systemic sclerosis severity scale was established. (3) Results: In the studied groups, comparable periodontitis rates of 88.68% and 85.85%, respectively, were identified. There were no significant differences in the severity of periodontitis among different systemic sclerosis severity, or in the positivity for anti-centromere and anti-SCL70 antibodies. Musculoskeletal lesions were significantly more common in stage III/IV periodontitis (n = 33, 86.84%) than in those in stage I/II (n = 1, 100%, and n = 3, 37.5%, respectively) (p = 0.007). Comparable levels of the inflammatory mediators were displayed by the two groups. There were no significant differences in calprotectin and psoriasin levels between diffuse and limited forms of systemic sclerosis. (4) Conclusions: Within the limitations of the current study, no associations between systemic sclerosis and periodontitis, or between their risk factors, could be proven.
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  • 文章类型: Journal Article
    背景:硬皮病是一种罕见的复杂疾病。它影响生活质量,严重影响皮肤和内部器官的广泛并发症,并且没有明确的治疗方法。本研究旨在探讨自我管理计划对硬皮病患者生活质量的影响。
    方法:这是一项临床试验,将54例硬皮病患者随机分为两组,每组27例(实验组和对照组)。使用系统性硬化症问卷收集数据。为期三个月,每天通过手机应用程序(WhatsApp)向实验组发送自我管理程序。统计分析在社会科学统计软件包V21中进行。
    结果:Wilcoxon符号秩检验显示,实验组的平均总体生活质量评分在方案实施后显示出显着提高(P值:0.00)。对照组干预后生活质量平均评分也明显下降(P值:0.00)。Mann-WhitneyU检验显示,干预前两组患者的总体生活质量评分无显著差异(P值:0.31);实施自我管理计划后,两组间差异有统计学意义(P值:0.00).
    结论:根据结果,自我管理方案有助于提高硬皮病患者的生活质量。
    BACKGROUND: Scleroderma is a rare disease with complex disorders. It affects the quality of life with severe impacts on the skin and extensive complications in the internal organs, and does not have a definitive treatment. This study aimed to investigate the effect of a self-management program on the quality of life of patients with scleroderma.
    METHODS: This was a clinical trial in which 54 patients with scleroderma were randomly divided into two groups of 27 each (experimental and control groups). The data were collected using the Systemic Sclerosis Questionnaire. A self-management program was sent to the experimental group via a mobile phone application (WhatsApp) every day for three months. Statistical analysis was performed in Statistical Package for the Social Sciences V21.
    RESULTS: The Wilcoxon signed-rank test showed that the average overall quality of life score of the experimental group showed a significant increase after the implementation of the program (P value: 0.00). The average overall quality of life score of the control group also significantly declined after the intervention (P value: 0.00). The Mann-Whitney U test revealed that there was no significant difference in the overall quality of life score of the two groups before the intervention (P value: 0.31); however, after the implementation of the self-management program, a significant difference was observed between the two groups (P value: 0.00).
    CONCLUSIONS: According to the results, the self-management program can help improve the quality of life of patients with scleroderma.
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  • 文章类型: Journal Article
    目的:系统性硬皮病(SSc)患者常出现牙齿早衰。这项研究的目的是通过放射学研究SSc患者牙科植入物的骨丢失,并将其与健康患者文献中的数据进行比较。
    方法:在全景和口内X线片上独立确定植入物的内侧和远端骨水平变化。在三个不同的评估者确定可评估性后,对它们进行了双重检查。科恩的卡帕被用来检验评估者间的可靠性。使用线性回归分析将患者视为随机效应植入物来估计平均骨丢失,并在不同时间点的预定义植入物区域以及植入物的内侧和远侧分别进行。
    结果:使用根尖周和全景X线片分析了61个植入物的内侧和远端骨水平变化。总的来说,在内侧和远端区域均可评估来自18例患者的114张X射线照片。经过60个月的最长观察期,平均种植体周围骨丢失在远端为1.68mm(范围:0.83至2.54mm),在右后下颌骨的内侧为1.65mm(范围:0.81至2.48mm)(区域44至47[FDI编号系统]),而在左后上颌骨(区域24至27),种植体周围骨丢失在远端为0.61mm(范围:0.32~0.91mm),在近端为0.59mm(范围:0.16~1.03mm).术后60个月平均骨丢失为1.05mm(范围:0.85至1.25mm)。
    结论:SSc患者植入物的边缘性骨丢失与健康受试者收集的文献数据相当。
    OBJECTIVE: Patients with systemic scleroderma (SSc) often suffer from premature tooth loss. The aim of this study was to radiologically investigate bone loss at dental implants in patients with SSc and compare it with data from the literature on healthy patients.
    METHODS: Mesial and distal bone level changes at implants were independently determined on panoramic and intraoral radiographs. They were double-checked after determination of evaluability by three different raters. Cohen\'s kappa was used to test for interrater reliability. Mean bone loss was estimated using linear regression analysis considering the patient as a random-effect implant and performed separately in predefined implant regions for different time points and for the mesial and distal sides of the implants.
    RESULTS: Mesial and distal bone level changes were analyzed in 61 implants using periapical and panoramic radiographs. In total, 114 radiographs from 18 patients were evaluable in both the mesial and distal regions. After a maximum observation period of 60 months, the mean peri-implant bone loss was 1.68 mm (range: 0.83 to 2.54 mm) at the distal aspect and 1.65 mm (range: 0.81 to 2.48 mm) at the mesial aspect in the right posterior mandible (region 44 to 47 [FDI numbering system]), whereas in the left posterior maxilla (regions 24 to 27), the mean peri-implant bone loss was 0.61 mm (range: 0.32 to 0.91 mm) at the distal aspect and 0.59 mm (range: 0.16 to 1.03 mm) at the mesial aspect. The mean bone loss 60 months after surgery was 1.05 mm (range: 0.85 to 1.25 mm).
    CONCLUSIONS: Marginal bone loss at implants in patients with SSc is comparable to data from the literature collected in healthy subjects.
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  • 文章类型: Clinical Trial
    面部和领口毛细血管扩张对系统性硬皮病(SSc)患者的生活质量有低估但重要的影响。这个单中心,prospective,开放标签,我们对21例SSc患者进行了患者内部比较研究.患者相隔8周接受4次PDL治疗。在最后一次会议结束后2个月,几个评估者进行了最后的四重评估,基于以下标准:毛细血管扩张数量的变化;主观改善评分(LINKERT量表);对生活质量的影响(QoL;SKINDEX评分);视觉模拟疼痛量表;不良反应(AE),包括PDL诱导的紫癜的治疗停止和患者满意度。在方案结束时,平均毛细血管扩张数量减少了5(32%)。18例患者(85.7%)报告改善或显著改善,而专家委员会的比例为73.81%。即刻疼痛(平均值=3.4/10)略低于总体疼痛(平均值=4.6/10)。10名患者(47%)至少经历了一次不良事件(渗出/结皮,水肿,表皮起泡),包括PDL诱导的紫癜3例(14%)。AEs大多是短暂的(<1周)和轻度的(CTCAE1级)。治疗后所有QoL参数均有所改善,85%的患者感到满意。
    Facial and neckline telangiectasias have an underestimated yet important impact on quality of life of patients with systemic scleroderma (SSc). This monocentric, prospective, open-label, intra-patient comparative study was conducted in 21 consecutive patients with SSc. Patients underwent 4 sessions of PDL 8 weeks apart. A final quadruple assessment was performed by several raters 2 months after the last session, based on the following criteria: change in telangiectasia number; subjective improvement score (LINKERT scale); impact on the quality of life (QoL; SKINDEX score); visual analog pain scale; adverse effects (AEs), including treatment discontinuation for PDL-induced purpura and patient satisfaction. The mean telangiectasia number decreased by 5 (32%) at the end of the protocol. Eighteen patients (85.7%) reported an improvement or a strong improvement, versus 73.81% for the expert committee. Immediate session pain (mean = 3.4/10) was slightly less than overall pain (mean = 4.6/10). Ten patients (47%) experienced at least one AE (oozing/crusts, edema, epidermal blistering), including PDL-induced purpura in 3 patients (14%). AEs were mostly transient (<1 week) and mild (CTCAE grade 1). All QoL parameters improved after treatment, and 85% of patients were satisfied.
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