Connective Tissue Diseases

结缔组织疾病
  • 文章类型: Journal Article
    本系统综述的目的是公开自身免疫性疾病及其医学治疗对牙种植体存活和成功的影响。
    使用MEDLINE(PubMed)进行了文献检索,Cochrane图书馆和Embase直到12月6日,2021年。对进行植入治疗的自身免疫性疾病患者的任何临床研究都是合格的。使用纽卡斯尔-渥太华量表评估纳入研究的质量。对于每个自身免疫性疾病组,数据综合分为三组:1)自身免疫性疾病的总体结果,2)相应对照组的总体结果和3)伴随自身免疫疾病的自身免疫疾病的总体结果(组1的亚组)。使用描述性统计。
    在4,865篇已确定的文章中,67可以包括在内,主要包括病例报告和回顾性研究,总体质量较低。在17.7至68.1个月的加权平均随访后,患者和植入物水平的植入物存活率为50%至100%。偶尔报道植入成功。有关免疫抑制药物的数据报告过于异质,无法进行详细分析。
    总的来说,据报道,自身免疫性疾病患者的植入物存活率很高。然而,已确定的研究的特点是质量低.由于报告不均匀,因此无法得出有关植入成功和免疫抑制剂作用的结论。
    UNASSIGNED: The purpose of this systematic review is to disclose the impact of autoimmune diseases and their medical treatment on dental implant survival and success.
    UNASSIGNED: A literature search was conducted using MEDLINE (PubMed), The Cochrane Library and Embase up to December 6th, 2021. Any clinical study on patients with an autoimmune disease in whom implant therapy was performed was eligible. The quality of included studies was assessed using the Newcastle-Ottawa Scale. For each autoimmune disease group, data synthesis was divided into three groups: 1) overall results of the autoimmune disease, 2) overall results of corresponding control groups and 3) overall results of the autoimmune disease with a concomitant autoimmune disease (a subgroup of group 1). Descriptive statistics were used.
    UNASSIGNED: Of 4,865 identified articles, 67 could be included and mainly comprising case reports and retrospective studies with an overall low quality. Implant survival rate was 50 to 100% on patient and implant level after a weighted mean follow-up of 17.7 to 68.1 months. Implant success was sporadically reported. Data on immunosuppressive medication were too heterogeneously reported to allow detailed analysis.
    UNASSIGNED: Overall, a high implant survival rate was reported in patients with autoimmune diseases. However, the identified studies were characterized by a low quality. No conclusions could be made regarding implant success and the effect of immunosuppressants due to heterogeneous reporting.
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  • 文章类型: Journal Article
    间质性肺病(ILD)由一组肺疾病组成,其特征在于与经常导致终末期呼吸衰竭的进行性呼吸困难相关的肺实质的炎症和/或纤维化。在美国,ILD影响约650000人,每年造成约25000至30000人死亡。
    ILD最常见的形式是特发性肺纤维化(IPF),约占所有ILD病例的三分之一,过敏性肺炎,占ILD病例的15%,结缔组织病(CTD),占ILD病例的25%。ILD通常表现为劳累时呼吸困难。大约30%的ILD患者报告咳嗽。胸部计算机断层扫描对诊断ILD亚型(如IPF)的敏感性约为91%,特异性约为71%。生理评估提供了重要的预后信息。与FVC无变化相比,12个月内用力肺活量(FVC)下降5%与死亡率增加约2倍有关。使用尼达尼布或吡非尼酮的抗纤维化治疗可使IPF患者的年FVC下降约44%至57%。硬皮病相关ILD,以及任何原因的进行性肺纤维化患者。对于结缔组织疾病相关的ILD,免疫调节治疗,比如托珠单抗,利妥昔单抗,和霉酚酸酯,在12个月的随访中,FVC可能会减缓下降甚至改善。结构化运动疗法可减少呼吸困难患者的症状并改善6分钟步行测试距离。氧气可减少ILD患者的症状并改善其生活质量,这些患者在6分钟的步行测试中饱和度低于88%。肺移植可以改善终末期ILD患者的症状并解决呼吸衰竭。肺移植后,ILD患者的中位生存期为5.2~6.7年,而未接受肺移植的晚期ILD患者的中位生存期小于2年.高达85%的终末期纤维化ILD个体发展为肺动脉高压。在这些患者中,吸入曲前列环素治疗可改善步行距离和呼吸道症状。
    间质性肺病通常表现为劳累时呼吸困难,并可进展为呼吸衰竭。一线治疗包括尼达尼布或吡非尼酮治疗IPF,霉酚酸酯治疗结缔组织疾病引起的ILD。晚期ILD患者应考虑肺移植。在ILD患者中,运动训练提高了6分钟步行测试距离和生活质量。
    Interstitial lung disease (ILD) consists of a group of pulmonary disorders characterized by inflammation and/or fibrosis of the lung parenchyma associated with progressive dyspnea that frequently results in end-stage respiratory failure. In the US, ILD affects approximately 650 000 people and causes approximately 25 000 to 30 000 deaths per year.
    The most common forms of ILD are idiopathic pulmonary fibrosis (IPF), which accounts for approximately one-third of all cases of ILD, hypersensitivity pneumonitis, accounting for 15% of ILD cases, and connective tissue disease (CTD), accounting for 25% of ILD cases. ILD typically presents with dyspnea on exertion. Approximately 30% of patients with ILD report cough. Thoracic computed tomography is approximately 91% sensitive and 71% specific for diagnosing subtypes of ILDs such as IPF. Physiologic assessment provides important prognostic information. A 5% decline in forced vital capacity (FVC) over 12 months is associated with an approximately 2-fold increase in mortality compared with no change in FVC. Antifibrotic therapy with nintedanib or pirfenidone slows annual FVC decline by approximately 44% to 57% in individuals with IPF, scleroderma associated ILD, and in those with progressive pulmonary fibrosis of any cause. For connective tissue disease-associated ILD, immunomodulatory therapy, such as tocilizumab, rituximab, and mycophenolate mofetil, may slow decline or even improve FVC at 12-month follow-up. Structured exercise therapy reduces symptoms and improves 6-minute walk test distance in individuals with dyspnea. Oxygen reduces symptoms and improves quality of life in individuals with ILD who desaturate below 88% on a 6-minute walk test. Lung transplant may improve symptoms and resolve respiratory failure in patients with end-stage ILD. After lung transplant, patients with ILD have a median survival of 5.2 to 6.7 years compared with a median survival of less than 2 years in patients with advanced ILD who do not undergo lung transplant. Up to 85% of individuals with end-stage fibrotic ILD develop pulmonary hypertension. In these patients, treatment with inhaled treprostinil improves walking distance and respiratory symptoms.
    Interstitial lung disease typically presents with dyspnea on exertion and can progress to respiratory failure. First-line therapy includes nintedanib or pirfenidone for IPF and mycophenolate mofetil for ILD due to connective tissue disease. Lung transplant should be considered for patients with advanced ILD. In patients with ILD, exercise training improves 6-minute walk test distance and quality of life.
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  • 文章类型: Review
    间质性肺病(ILD)可导致肺癌,这给鉴别诊断和综合治疗带来了极大的挑战。然而,肺显性结缔组织病(LD-CTD)相关ILD合并肺癌的临床特征尚未得到验证.我们报告了一名80岁的LD-CTD女性定期接受尼达尼布治疗的病例,该患者在反复病毒感染后出现进行性呼吸困难和低氧血症。她的胸部计算机断层扫描(CT)显示两下肺间质纤维化加重,右胸腔积液中度。临床医生应警惕对治疗反应差或ILD急性进展的患者的肺癌。现有的临床表现的鉴别诊断文献,成像,本研究对LD-CTD的治疗和预后进行了综述和讨论。
    Interstitial lung disease (ILD) can lead to lung cancer, which brings great challenges to differential diagnosis and comprehensive treatment. However, the clinical features of lung-dominant connective tissue disease (LD-CTD) related ILD combined with lung cancer has not been validated. We report the case of an 80-year-old woman with LD-CTD treated regularly with nintedanib who presented progressive dyspnoea and hypoxemia after recurrent viral infections. Her chest computed tomography (CT) showed aggravated interstitial fibrosis in both lower lungs with moderate right pleural effusion. Clinicians should be alert to lung cancer in patients who are experiencing poor responsiveness to treatment or acute progression of ILD. The available literatures about the differential diagnosis of clinical manifestations, imaging, treatment and prognosis of LD-CTD are reviewed and discussed in this study.
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  • 文章类型: Journal Article
    青少年系统性结缔组织病(JSCTD)是一组异质性的慢性自身免疫性疾病,与血脂异常和心血管风险增加相关。过去10年的研究,从2013年到2022年,收集了JSCTD中的血脂谱。选择了受JSCTD影响的儿童的血脂状况的不同研究,因为目的是分析这些患者的心血管风险和动脉粥样硬化的可能性,有时,皮质类固醇治疗和免疫抑制剂增加血脂异常的状态。一些研究表明,具有炎性底物的自身免疫性疾病也具有脂质分布异常和心血管风险增加。具体来说,已经发现青少年系统性结缔组织疾病和甘油三酯升高之间的关联,TC-C(总胆固醇),LDL-C(低密度脂蛋白),低HDL-C(高密度脂蛋白),并增加患心肌梗塞等疾病的风险,外周血管疾病,肺动脉高压,和心房颤动。另一方面,补充α-亚麻酸(ALA)也已被分析,在降低炎症参数方面具有积极的结果。如IL-6(白细胞介素-6),CRP(C反应蛋白),和空腹血糖,在血脂异常的受试者中。这些观察结果表明,补充ALA,omega-3前体,可以积极调节自身免疫性疾病患者的炎症状态和血脂异常。
    Juvenile Systemic Connective Tissue Diseases (JSCTD) are a heterogeneous group of chronic autoimmune diseases, associated with dyslipidemia and increased cardiovascular risk are related. Studies from the last 10 years, from 2013 to 2022, on lipid profiles in JSCTD were collected. Different studies on lipid profiles in children affected by JSCTD were selected, because the aim is to analyze the cardiovascular risk and the possibility of atherosclerosis in these patients in whom, sometimes, corticosteroid therapies and immunosuppressants increase the state of dyslipidemia. Several studies have shown that autoimmune diseases with an inflammatory substrate also share abnormalities in lipid profile and increased cardiovascular risk. Specifically, associations have been found between Juvenile Systemic Connective Tissue Diseases and elevated triglycerides, TC-C (Total Cholesterol), LDL-C (Low-Density Lipoprotein), low HDL-C (High-Density Lipoprotein), and increased risk of developing diseases such as myocardial infarction, peripheral vascular disease, pulmonary and arterial hypertension, and atrial fibrillation. Supplementation with alpha-linolenic acid (ALA) on the other hand has also been analyzed with positive results in reducing inflammatory parameters, such as IL-6 (Interleukin-6), CRP (C-reactive protein), and fasting glucose, in subjects with dyslipidemia. These observations suggest that supplementation with ALA, an omega-3 precursor, may positively modulate both the inflammatory status and dyslipidemic conditions in patients with autoimmune disorders.
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  • 文章类型: Journal Article
    虽然以特发性肺纤维化(IPF)为中心的特发性间质性肺炎(IIP)是最常见的间质性肺病(ILD),尤其是在老年人口,结缔组织病(CTD)相关的ILD是第二普遍的ILD。IPF的发病机制主要是纤维化,而其他ILD,特别是CTD-ILD,主要是炎症。因此,准确的诊断对于选择合适的治疗方法至关重要,如抗纤维化或免疫抑制剂。此外,一些IIP患者具有CTD相关特征,比如关节炎和皮肤出疹,但不符合任何CTD的标准,这被称为具有自身免疫特征的间质性肺炎(IPAF).IPAF与特发性非特异性间质性肺炎(iNSIP)和隐源性机化性肺炎(COP)密切相关。此外,iNSIP或NSIP伴OP重叠的患者在诊断IIP后经常发生多发性肌炎/皮肌炎.ILD急性加重,最常见的死因,IPF患者的发病率高于其他ILD患者。尽管CTD-ILD的急性加重发生率较低,类风湿性关节炎患者,显微镜下多血管炎,与其他CTD相比,系统性硬化症或CTD-ILD的急性加重。在这次审查中,每个IIP的特征,专注于CTD相关的签名,总结,并讨论了各种ILD患者的发病机制和改善预后的适当治疗方法。
    While idiopathic interstitial pneumonia (IIP) centering on idiopathic pulmonary fibrosis (IPF) is the most prevalent interstitial lung disease (ILD), especially in the older adult population, connective tissue disease (CTD)-related ILD is the second most prevalent ILD. The pathogenesis of IPF is primarily fibrosis, whereas that of other ILDs, particularly CTD-ILD, is mainly inflammation. Therefore, a precise diagnosis is crucial for selecting appropriate treatments, such as antifibrotic or immunosuppressive agents. In addition, some patients with IIP have CTD-related features, such as arthritis and skin eruption, but do not meet the criteria for any CTD, this is referred to as interstitial pneumonia with autoimmune features (IPAF). IPAF is closely associated with idiopathic nonspecific interstitial pneumonia (iNSIP) and cryptogenic organizing pneumonia (COP). Furthermore, patients with iNSIP or those with NSIP with OP overlap frequently develop polymyositis/dermatomyositis after the diagnosis of IIP. Acute exacerbation of ILD, the most common cause of death, occurs more frequently in patients with IPF than in those with other ILDs. Although acute exacerbation of CTD-ILD occurs at a low rate of incidence, patients with rheumatoid arthritis, microscopic polyangiitis, or systemic sclerosis experience more acute exacerbation of CTD-ILD than those with other CTD. In this review, the features of each IIP, focusing on CTD-related signatures, are summarized, and the pathogenesis and appropriate treatments to improve the prognoses of patients with various ILDs are discussed.
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  • 文章类型: Meta-Analysis
    背景:一些结果支持以下假设:一组属于视神经脊髓炎谱系障碍(NMOSD)诊断标准的病理可能与结缔组织疾病(CTD)并存,患者对自身免疫疾病具有高度易感性。然而,NMOSD与风湿病之间的关系值得进一步研究,以阐明这种共存的所有临床方面。我们设计了系统评价和比例荟萃分析来估计CTD和MNOSD之间的关联。目的是帮助规划最佳战略,为这些疾病实现最显著的公共卫生利益。
    方法:我们对2023年2月之前发表的文献进行了系统回顾,在四个数据库中进行了搜索:PubMed,WebofScience,Embase,和OVID。然后,我们进行了随机效应比例荟萃分析,并使用JoannaBriggs研究所核对表评估了纳入研究的偏倚风险.
    结果:文献检索得出了3176篇出版物的总体结果(来自PubMed,880来自WebofScience,634来自Embase,1390来自OVID)。其中,29人被纳入本系统综述。分析招募未选择的系统性红斑狼疮(SLE)和干燥综合征(SjS)患者的研究,NMOSD重叠的合并百分比为0.6%(95%置信区间[95%CI]:0.1%-1.4%,)和6.5%(95%CI:4.7-8.6),分别。纳入有神经系统症状的风湿病患者的研究报告NMOSD的百分比更高(即,在SjS患者中,合并比例为26.5%,95%CI:5.5-54.6%,找到了)。同样,招募NMOSD患者,我们发现SjS或SLE的合并百分比分别为7.0%和3.5%。
    结论:我们的研究发现,在诊断为SjS并有神经系统表现的女性风湿病患者和怀疑诊断为SjS的神经系统患者中,这两种疾病的共存更为常见。同样,在SLE中发现NMOSD较少,在混合性结缔组织疾病(MCTD)患者中很少发生。在风湿病学家和神经科医师的临床经验中应考虑这些因素,因为这两种疾病的早期诊断可能会影响免疫抑制治疗的时机和系统性残疾的预防。
    BACKGROUND: Several results support the hypothesis that a group of pathologies falling within the Neuromyelitis Optica Spectrum Disorders (NMOSD) diagnostic criteria may coexist with Connective Tissue Diseases (CTD) in patients with a high susceptibility to autoimmune conditions. However, the relationship between NMOSD and rheumatologic diseases deserves further investigations to clarify all clinical aspects of this coexistence. We designed a systematic review and a proportional meta-analysis to estimate the association between CTD and MNOSD, with the aim of helping to plan the best strategy to achieve the most significant public health benefit for these conditions.
    METHODS: We conducted a systematic review of the literature published until February 2023, searching in four databases: PubMed, Web of Science, EmBase, and OVID. Then, we conducted a random-effects proportional meta-analysis and assessed the risk of bias of the included studies using the Joanna Briggs Institute checklist.
    RESULTS: The literature search yielded an overall result of 3176 publications (272 from PubMed, 880 from Web of Science, 634 from EmBase and 1390 from OVID). Of these, 29 were included in this systematic review. Analyzing studies that recruited unselected patients with Systemic Lupus Erythematosus (SLE) and Sjogren Syndrome (SjS), the pooled percentages of NMOSD overlapping were 0.6% (95% Confidence Interval [95% CI]: 0.1%-1.4%,) and 6.5% (95% CI: 4.7-8.6), respectively. Studies enrolling rheumatologic patients with nervous system symptoms involvement reported higher percentage of NMOSD (i.e., among SjS patients, a pooled percentage of 26.5%, 95% CI: 5.5-54.6%, was found). Similarly, recruiting patients with NMOSD, we found pooled percentages of SjS or SLE respectively of 7.0% and 3.5%.
    CONCLUSIONS: Our research found that the coexistence of these two disorders was more frequent in female rheumatologic patients with a SjS diagnosis with neurological manifestations and in neurologic patients for whom a SjS diagnosis was suspected. Similarly, NMOSD are less frequently found in SLE and very rarely incident in Mixed Connective Tissue Disease (MCTD) patients. These considerations should be taken into account in clinical experience of rheumatologists and neurologists, since early diagnosis of both conditions may influence the timing of immunosuppressive therapy and the prevention of systemic disabilities.
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  • 文章类型: Systematic Review
    暂无摘要。
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  • 文章类型: Journal Article
    治疗结缔组织疾病相关肺动脉高压(CTD-PAH)的证据主要取决于随机对照试验(RCTs)的亚组或事后分析。因此,我们对报告CTD-PAH结局的RCT进行了荟萃分析.搜索PubMed和EMBASE的CTD-PAH治疗。选定的结果是功能类别(FC)变化,存活率,6分钟步行距离(6-MWD)临床恶化(CW),N-末端激素原BNP(NT-proBNP),肺血管阻力(PVR),平均肺动脉压(mPAP),右心房压力(RAP),和心脏指数(CI)。根据PRISMA指南进行荟萃分析,并在PROSPERO(CRD42020153560)中注册。纳入了对1837例患者进行的12项RCT。59%的诊断为系统性硬化症,SLE在20%,和其他CTD占21%。药物干预措施是依前列醇,曲前列环素,西地那非,他达拉非,波生坦,Macitentan,ambrisentan,Riociguat,和selexipag。在FC中,干预措施和安慰剂之间存在显着差异,6MWD,CW,PVR,RAP,赞成干预的CI。我们的分析表明,PAH治疗可将CW风险降低39%。研究组和对照组的短期生存率和平均血清NT-proBNP变化相似。CTD-PAH的治疗对临床和血流动力学结果有良好的影响,但对生存率和NT-proBNP水平没有影响。与之前关注6-MWD的荟萃分析不同,临床恶化的时间,和CW作为结果,此荟萃分析还报告了FC变化的汇总分析,血液动力学测量(RAP,PVR,CI),和NT-proBNP,其中一些对PAH具有预后价值。
    The evidence for the treatment of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) mostly depends on subgroup or post hoc analysis of randomized controlled trials (RCTs). Thus, we performed a meta-analysis of RCTs that reported outcomes for CTD-PAH. PubMed and EMBASE were searched for CTD-PAH treatment. The selected outcomes were functional class (FC) change, survival rates, 6-min walk distance (6-MWD), clinical worsening (CW), N-terminal prohormone BNP (NT-proBNP), pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), right atrial pressure (RAP), and cardiac index (CI). The meta-analysis was conducted according to the PRISMA guidelines and registered in PROSPERO (CRD42020153560). Twelve RCTs conducted with 1837 patients were included. The diagnoses were systemic sclerosis in 59%, SLE in 20%, and other CTDs in 21%. The pharmacological interventions were epoprostenol, treprostinil, sildenafil, tadalafil, bosentan, macitentan, ambrisentan, riociguat, and selexipag. There was a significant difference between interventions and placebo in FC, 6MWD, CW, PVR, RAP, and CI that favored intervention. Our analysis showed a 39% reduction in the CW risk with PAH treatment. The short-term survival rates and mean serum NT-proBNP changes were similar between the study and control groups. Treatment for CTD-PAH had favorable effects on clinical and hemodynamic outcomes but not on survival and NT-proBNP levels. Different from the previous meta-analyses that focused on 6-MWD, time to clinical worsening, and CW as outcomes, this meta-analysis additionally reports the pooled analysis of change in FC, hemodynamic measurements (RAP, PVR, CI), and NT-proBNP, some of which have prognostic value for PAH.
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  • 文章类型: Case Reports
    肺动脉高压(PAH),特发性肺纤维化(IPF),和硬皮病(SSc)是三种相互关联的医学疾病,可导致显著的发病率和死亡率。肺动脉高压,肺部有高血压,会导致心力衰竭和其他并发症。特发性肺纤维化,一种以肺组织结疤为特征的进行性肺病,会导致呼吸困难和氧合受损。硬皮病,一种自身免疫性疾病,会引起皮肤和内脏器官的增厚和硬化,包括肺,导致肺纤维化和高血压。目前,这些情况都没有治愈方法。然而,早期发现和适当的管理可以改善患者的生活质量和预后。这篇综述集中在SSc患者的PH和IPF。提供有关原因的信息,症状,以及这些疾病的治疗,与说明性图像一起。它还提供了相关医疗条件的概述:PH,IPF,SSC。它强调了早期发现和适当管理以改善患者生活质量和预后的重要性。
    Pulmonary arterial hypertension (PAH), idiopathic pulmonary fibrosis (IPF), and scleroderma (SSc) are three interrelated medical conditions that can result in significant morbidity and mortality. Pulmonary hypertension, a condition marked by high blood pressure in the lungs, can lead to heart failure and other complications. Idiopathic pulmonary fibrosis, a progressive lung disease characterised by scarring of lung tissue, can cause breathing difficulties and impaired oxygenation. Scleroderma, an autoimmune disease, can induce thickening and hardening of the skin and internal organs, including the lungs, leading to pulmonary fibrosis and hypertension. Currently, there is no cure for any of these conditions. However, early detection and proper management can improve the quality of life and prognosis of a patient. This review focusses on PH and IPF in patients with SSc, providing information on the causes, symptoms, and treatment of these conditions, together with illustrative images. It also provides an overview of interrelated medical conditions: PH, IPF, and SSc. It emphasises the importance of early detection and proper management to improve patient quality of life and prognosis.
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  • 文章类型: Journal Article
    目的:自身免疫性大疱性疾病,结缔组织疾病,血管炎代表一组严重的罕见皮肤病。虽然糖皮质激素和免疫抑制剂是这些疾病的标准治疗方法,由于不良副作用,它们的功效有限,表明需要替代方法。生物制剂已用于管理一些罕见的皮肤病。然而,生物制剂的使用与担忧有关,如感染风险和高成本,促使人们寻求有效和具有成本效益的替代品。这项研究讨论了与托法替尼相关的安全性问题及其在治疗罕见皮肤病中的潜力。
    方法:这篇叙述性综述集中于托法替尼的药效学特性及其对JAK/STAT通路的影响。此外,我们全面讨论了托法替尼对每种严重罕见皮肤病的疗效和作用机制。
    结果:已经讨论了托法替尼在治疗严重罕见皮肤病中的作用,揭示了其作为一种治疗方式的有希望的前景。在使用托法替尼治疗的患者中,很少有严重不良事件的报告。
    结论:我们探索了作用机制,功效,和托法替尼的安全性考虑,发现它可以用作罕见皮肤病的治疗选择。然而,需要多中心临床研究来证实JAK抑制剂的有效性和安全性.
    OBJECTIVE: Autoimmune bullous diseases, connective tissue diseases, and vasculitis represent a group of severe rare skin diseases. While glucocorticoids and immunosuppressive agents serve as standard treatments for these diseases, their efficacy is limited due to adverse side effects, indicating the need for alternative approaches. Biologics have been used in the management of some rare skin diseases. However, the use of biologics is associated with concerns, such as infection risk and high costs, prompting the quest for efficacious and cost-effective alternatives. This study discusses the safety issues associated with tofacitinib and its potential in treating rare skin diseases.
    METHODS: This narrative review focuses on the pharmacodynamic properties of tofacitinib and its impact on the JAK/STAT pathway. In addition, we present a comprehensive discussion of the effects and mechanism of action of tofacitinib for each severe rare skin disease.
    RESULTS: This role of tofacitinib in treating severe rare skin diseases has been discussed, shedding light on its promising prospects as a treatment modality. Few reports of serious adverse events are available in patients treated with tofacitinib.
    CONCLUSIONS: We explored the mechanism of action, efficacy, and safety considerations of tofacitinib and found that it can be used as a treatment option for rare skin diseases. However, multicenter clinical studies are needed to confirm the efficacy and safety of JAK inhibitors.
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