Chronic nodular prurigo

慢性结节性痒疹
  • 文章类型: Journal Article
    目的:结节性痒疹(PN)是一种皮肤疾病,其特征是皮肤结节严重发痒,与重要的医疗保健资源利用(HCRU)有关。这项研究旨在评估英格兰PN总体和中度至重度PN(MSPN)患者的HCRU。
    方法:这项回顾性队列研究使用了来自英国临床实践研究数据链和医院事件统计的数据。在主要分析中,将轻度PN(MiPN)患者与MSPN患者的年龄和性别进行匹配。患者在2007年4月1日至2019年3月1日期间纳入研究。计算了全因HCCU,包括初级和二级保健接触者和费用(成本年2022)。
    结果:在23,522名确定的患者中,8,933符合纳入标准,与2,479名PN患者的主要匹配队列。随访期间,MSPN组和MiPN组的匹配队列初级护理访视次数分别为21.27/患者年(PPY)和11.35PPY.MSPN和MiPN组的任何门诊量为10.72PPY和4.87PPY,分别。MSPN和MiPN组的门诊皮肤科访视为1.96PPY和1.14PPY,分别。
    结论:PN,尤其是MSPN,在英国有很高的HCCU负担,强调需要新的和改进的疾病管理治疗。
    Purpose: Prurigo nodularis (PN) is a skin disease characterized by intensely itchy skin nodules and is associated with a significant healthcare resource utilization (HCRU). This study aimed to estimate the HCRU of patients in England with PN overall and moderate-to-severe PN (MSPN) in particular.
    Methods: This retrospective cohort study used data from the Clinical Practice Research Datalink and Hospital Episode Statistics in England. Patients with Mild PN (MiPN) were matched to patients with MSPN by age and gender for the primary analysis. Patients were enrolled in the study between 1st April 2007 and 1st March 2019. All-cause HCRU was calculated, including primary and secondary care contacts and costs (cost-year 2022).
    Results: Of 23,522 identified patients, 8,933 met the inclusion criteria, with a primary matched cohort of 2,479 PN patients. During follow up, the matched cohort\'s primary care visits were 21.27 per patient year (PPY) for MSPN group and 11.35 PPY for MiPN group. Any outpatient visits were 10.72 PPY and 4.87 PPY in MSPN and MiPN groups, respectively. Outpatient dermatology visits were 1.96 PPY and 1.14 PPY in MSPN and MiPN groups, respectively.
    Conclusion: PN, especially MSPN, has a high HCRU burden in England, highlighting the need for new and improved disease management treatments.
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  • 文章类型: English Abstract
    BACKGROUND: Despite the high burden in patients with chronic prurigo (CPG), the first and so far only approved systemic therapy for this disease, dupilumab, has only been available since 2022. Therefore, treatment is mostly based on expert recommendations for off-label therapies. We aim to provide an overview of current therapies and possible future therapeutic drugs for CPG patients, which are currently in clinical trials.
    METHODS: For this review, a systematic literature and clinical trial search was conducted via PubMed and Clinical Trials using the terms \"chronic prurigo\", \"chronic nodular prurigo\", \"prurigo nodularis\" and \"therapy\".
    CONCLUSIONS: Multiple new therapeutic agents are currently under investigation in clinical trials, providing promising results for future treatment options. Moreover, an annotated checklist was developed recently to improve therapeutic decision-making in daily clinical practice with CPG patients.
    UNASSIGNED: HINTERGRUND: Trotz des hohen Leidensdrucks der Patienten mit chronischer Prurigo (CPG) steht erst seit 2022 mit Dupilumab die erste und bislang einzige zugelassene systemische Therapie dieser Erkrankung zur Verfügung. Mehrheitlich stützt sich die Therapie der CPG daher weiterhin auf Expertenempfehlungen zu Off-label-Therapien. Ziel dieser Arbeit ist es, einen Überblick über aktuelle therapeutische Optionen der CPG sowie einen Ausblick auf potenzielle zukünftige Therapeutika zu geben, die sich derzeit in klinischer Erprobung befinden.
    METHODS: Für diese Übersichtsarbeit wurde eine systematische Literatur- und klinische Studienrecherche über PubMed und Clinical Trials mit den Begriffen „Chronic Prurigo“, „Chronic Nodular Prurigo“, „Prurigo nodularis“ und „Therapy“ durchgeführt.
    UNASSIGNED: Es befindet sich eine Vielzahl neuer Substanzen in verschiedenen Stadien der klinischen Erprobung mit vielversprechenden Ergebnissen, hierunter der IL(Interleukin)-31-Antikörper Nemolizumab. Zudem wurde kürzlich eine praxisorientierte Checkliste als Entscheidungshilfe für die Einleitung einer Systemtherapie der CPG entwickelt.
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  • 文章类型: Practice Guideline
    慢性结节性痒疹(CNP)是一种慢性皮肤病,其特征是存在慢性瘙痒和瘙痒性结节性病变。这项研究的目的是在非系统文献综述和CNP临床诊断算法的基础上,在一组专家之间达成共识。所得到的算法被构造为3个块:1)对具有可能的CNP诊断的患者的早期识别;2)CNP的诊断和评估;以及3)CNP的分类(潜在原因或相关合并症的识别)。我们认为这种临床算法可以促进CNP患者的正确诊断。此外,它提高了人们对CNP需要多学科方法和具体治疗的认识,做出更好的治疗决策的最重要步骤。
    Chronic nodular prurigo (CNP) is a chronic dermatological disease characterized by the presence of chronic pruritus and pruritic nodular lesions. The aim of this study was to reach consensus among a group of experts based on a non-systematic literature review and an algorithm for the clinical diagnosis of CNP. The resulting algorithm is structured in 3 blocks: 1) early identification of the patient with a possible diagnosis of CNP; 2) diagnosis and assessment of CNP; and 3) categorization of CNP (identification of the underlying causes or associated comorbidities). We believe that this clinical algorithm can facilitate the correct diagnosis of patients with CNP. Additionally, it raises awareness on the need for a multidisciplinary approach and specific treatment of CNP, steps of paramount importance to make better therapeutic decisions.
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  • 文章类型: Journal Article
    背景:慢性结节性痒疹(CNPG)是一种慢性炎症性皮肤病,由慢性瘙痒-划痕周期维持,可能源于神经免疫失调。这种情况可能与某些患者的特应性有关,并且现在有来自阻断2型细胞因子如IL-4、IL-13和IL-31的有希望的治疗结果。然而,潜在的病理机制以及与特应性的分子关系仍然不明确。
    方法:我们使用单细胞RNA测序结合T细胞受体测序,将CNPG患者的皮肤病变与特应性皮炎(AD)和健康对照(HC)个体进行比较。
    结果:我们在CNPG和AD中发现了2型免疫偏移,如表达IL13的CD4+辅助性T细胞所证明。然而,只有AD有额外的,寡克隆扩增的CD8A+IL9R+IL13+细胞毒性T细胞群,免疫激活途径在AD中高度上调,但在CNPG中就更少了。相反,CNPG显示细胞外基质组织的特征,胶原蛋白合成,和纤维化,包括CXCL14-IL24+分泌型乳头状成纤维细胞的独特群体。除了已知的瘙痒介质,如IL31和制瘤素M(OSM),与AD和HC相比,我们还检测到CNPG病变的成纤维细胞中神经介蛋白B(NMB)的水平升高,在一些神经末梢可检测到NMB受体。
    结论:这些数据表明,CNPG并不具有通常在AD中发现的强大的疾病特异性免疫激活途径,但其特征是基质重塑机制上调,可能直接影响瘙痒纤维。
    Chronic nodular prurigo (CNPG) is an inflammatory skin disease that is maintained by a chronic itch-scratch cycle likely rooted in neuroimmunological dysregulation. This condition may be associated with atopy in some patients, and there are now promising therapeutic results from blocking type 2 cytokines such as IL-4, IL-13, and IL-31.
    This study aimed to improve the understanding of pathomechanisms underlying CNPG as well as molecular relationships between CNPG and atopic dermatitis (AD).
    We profiled skin lesions from patients with CNPG in comparison with AD and healthy control individuals using single-cell RNA sequencing combined with T-cell receptor sequencing.
    We found type 2 immune skewing in both CNPG and AD, as evidenced by CD4+ helper T cells expressing IL13. However, only AD harbored an additional, oligoclonally expanded CD8A+IL9R+IL13+ cytotoxic T-cell population, and immune activation pathways were highly upregulated in AD, but less so in CNPG. Conversely, CNPG showed signatures of extracellular matrix organization, collagen synthesis, and fibrosis, including a unique population of CXCL14-IL24+ secretory papillary fibroblasts. Besides known itch mediators such as IL31 and oncostatin M, we also detected increased levels of neuromedin B in fibroblasts of CNPG lesions compared with AD and HC, with neuromedin B receptors detectable on some nerve endings.
    These data show that CNPG does not harbor the strong disease-specific immune activation pathways that are typically found in AD but is rather characterized by upregulated stromal remodeling mechanisms that might have a direct impact on itch fibers.
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  • 文章类型: Journal Article
    慢性瘙痒,瘙痒持续超过六周,对患者的健康和生活质量构成重大挑战。这是访问皮肤科医生和全科医生的常见原因,可能是由一系列条件引起的,包括慢性肾病或肝病等全身性疾病,恶性肿瘤,以及神经病症和皮肤病,如特应性皮炎。慢性瘙痒通常不会与疾病的过程平行发展,并且可以成为其自身的实体,必须止痒治疗,即使根本原因已经在治疗中。根据慢性瘙痒的病因,最近已经分析了发病机理中的不同途径,随后开发了新的治疗方法,并在随机对照试验中进行了测试.本文讨论了这些研究的最新结果,并重点介绍了如何最好地管理慢性瘙痒患者的医疗保健。
    Chronic pruritus (CP) (ie, itch that persists for more than 6 weeks) poses significant challenges to patients\' health and quality of life. It is a common reason for visits to dermatologists and general practitioners and can be caused by a range of conditions, including systemic diseases such as chronic kidney disease or liver diseases, malignancies, neuropathic conditions, and dermatoses such as atopic dermatitis. CP often does not develop in parallel with the course of the disease and can become an entity of its own, which must be treated with antipruritic drugs, even if the underlying cause is already under therapy. Depending on the etiology of CP, different pathways in the pathogenesis have been analyzed recently, following which new treatments have been developed and tested in randomized controlled trials. This article discusses the recent results of these studies and highlights how best to manage health care for patients with CP.
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  • 文章类型: Journal Article
    慢性结节性痒疹(CNPG)是一种顽固性慢性瘙痒疾病,影响生活质量。它可以由多种病因引发,如特应性皮炎,糖尿病,和慢性肾脏疾病。CNPG的机制复杂,涉及皮肤的相互作用,免疫,和神经系统。多种免疫细胞,包括嗜酸性粒细胞,中性粒细胞,T细胞,巨噬细胞,肥大细胞浸润了CNPG的皮损,这引发了炎症细胞因子和瘙痒原的释放。此外,免疫细胞与激活的周围感觉神经纤维之间的相互作用通过神经递质引起皮肤神经炎症和顽固性瘙痒。CNPG的这种瘙痒-抓痒的恶性循环导致疾病恶化。CNPG难以用传统疗法治疗。最近,CNPG在炎症和瘙痒传播的病理生理学方面都取得了很大进展。在这次审查中,我们总结了CNPG的最新机制和新疗法。
    Chronic nodular prurigo (CNPG) is a recalcitrant chronic itchy disorder that affects the quality of life. It can be triggered by multiple etiologies, such as atopic dermatitis, diabetes, and chronic renal diseases. The mechanisms of CNPG are complicated and involved the interaction of the cutaneous, immune, and nervous systems. Diverse immune cells, including eosinophils, neutrophils, T cells, macrophages, and mast cells infiltrated the lesional skin of CNPG, which initiated the inflammatory cytokines and pruritogens release. In addition, the interaction between the immune cells and activated peripheral sensory nerve fibers by neurotransmitters caused neuroinflammation in the skin and intractable itch. This itch-scratch vicious cycle of CNPG results in disease exacerbation. CNPG is difficult to treat with traditional therapies. Recently, great advances have been made in the pathophysiology of both inflammation and pruritus transmission in CNPG. In this review, we summarize the updated mechanisms and novel therapies for CNPG.
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  • 文章类型: Journal Article
    目的:慢性结节性痒疹(CNPG)是一种最新定义且目前未被诊断的疾病,具有多种原因。它与多种合并症有关,随着对其发病机制的进一步了解,其管理和治疗有所改善。这项研究的目的是描述我们对一系列CNPG患者的经历。
    方法:单中心,观察,回顾性研究2009年至2021年在三级医院皮肤科就诊的CNPG患者的社会人口统计学和临床特征。
    结果:我们纳入了74例患者,主要是女性(63.5%),平均年龄57岁.总的来说,39.2%的患者有合并的皮肤状况,主要是特应性皮炎(62%)。其他合并症包括内分泌失调(54.1%),心血管疾病(44.4%),和精神疾病(36.5%)。在70%的病例中,皮肤活检有助于确认临床诊断。平均免疫球蛋白E水平高于正常值(516IU/mL),不管特应性易感性。平均而言,患者接受了三种治疗,最常见的选择是甲氨蝶呤,抗组胺药,以及局部和口服皮质类固醇。甲氨蝶呤是最有效的选择之一。
    结论:CNPG是一种与多种合并症相关的复杂疾病。这需要多学科的方法,中心的皮肤科医生.传统的治疗方法可能是不够的。
    OBJECTIVE: Chronic nodular prurigo (CNPG) is a recently defined and currently underdiagnosed disease with a variety of causes. It is associated with multiple comorbidities, and its management and treatment have improved with a better understanding of its pathogenesis. The aim of this study was to describe our experience with a series of patients with CNPG.
    METHODS: Single-center, observational, retrospective study of the sociodemographic and clinical characteristics of patients with CNPG seen at the dermatology department of a tertiary care hospital between 2009 and 2021.
    RESULTS: We included 74 patients, mostly women (63.5%), with a mean age of 57 years. Overall, 39.2% of patients had a concomitant skin condition, mainly atopic dermatitis (62%). Other comorbidities included endocrine disorders (54.1%), cardiovascular disease (44.4%), and psychiatric disorders (36.5%). Skin biopsy helped confirm the clinical diagnosis in 70% of cases. The mean immunoglobulin E level was higher than normal (516IU/mL), regardless of atopic predisposition. On average, patients received three treatments, the most common choices being methotrexate, antihistamines, and topical and oral corticosteroids. Methotrexate was among the most effective options.
    CONCLUSIONS: CNPG is a complex disease associated with multiple comorbidities. It requires a multidisciplinary approach, with the dermatologist at the center. Classical treatment approaches are probably insufficient.
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  • 文章类型: Journal Article
    目的:慢性结节性痒疹(CNPG)是一种最新定义且目前未被诊断的疾病,具有多种原因。它与多种合并症有关,随着对其发病机制的进一步了解,其管理和治疗有所改善。这项研究的目的是描述我们对一系列CNPG患者的经历。
    方法:单中心,观察,回顾性研究2009年至2021年在三级医院皮肤科就诊的CNPG患者的社会人口统计学和临床特征。
    结果:我们纳入了74例患者,主要是女性(63.5%),平均年龄57岁.总的来说,39.2%的患者有合并的皮肤状况,主要是特应性皮炎(62%)。其他合并症包括内分泌失调(54.1%),心血管疾病(44.4%),和精神疾病(36.5%)。在70%的病例中,皮肤活检有助于确认临床诊断。平均免疫球蛋白E水平高于正常值(516IU/mL),不管特应性易感性。平均而言,患者接受了3种治疗,最常见的选择是甲氨蝶呤,抗组胺药,以及局部和口服皮质类固醇。甲氨蝶呤是最有效的选择之一。
    结论:CNPG是一种与多种合并症相关的复杂疾病。这需要多学科的方法,中心的皮肤科医生.传统的治疗方法可能是不够的。
    OBJECTIVE: Chronic nodular prurigo (CNPG) is a recently defined and currently underdiagnosed disease with a variety of causes. It is associated with multiple comorbidities, and its management and treatment have improved with a better understanding of its pathogenesis. The aim of this study was to describe our experience with a series of patients with CNPG.
    METHODS: Single-center, observational, retrospective study of the sociodemographic and clinical characteristics of patients with CNPG seen at the dermatology department of a tertiary care hospital between 2009 and 2021.
    RESULTS: We included 74 patients, mostly women (63.5%), with a mean age of 57 years. Overall, 39.2% of patients had a concomitant skin condition, mainly atopic dermatitis (62%). Other comorbidities included endocrine disorders (54.1%), cardiovascular disease (44.4%), and psychiatric disorders (36.5%). Skin biopsy helped confirm the clinical diagnosis in 70% of cases. The mean immunoglobulin E level was higher than normal (516 IU/mL), regardless of atopic predisposition. On average, patients received 3 treatments, the most common choices being methotrexate, antihistamines, and topical and oral corticosteroids. Methotrexate was among the most effective options.
    CONCLUSIONS: CNPG is a complex disease associated with multiple comorbidities. It requires a multidisciplinary approach, with the dermatologist at the center. Classical treatment approaches are probably insufficient.
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  • 文章类型: Journal Article
    背景:慢性结节性痒疹(CNPG)是一种慢性,炎症性皮肤病,以强烈和衰弱的瘙痒为特征。病理生理学尚未完全了解,这种情况很难在没有靶向治疗的情况下治疗。本系统综述的目的是回顾非特应性CNPG治疗的证据,并对结果进行荟萃分析。
    结论:我们对有关非特应性CNPG治疗效果的文献进行了系统综述。由于随机对照试验(RCT)和病例系列很少,不幸的是,文献太稀少,无法对结果进行荟萃分析.相反,我们彻底报道了3项现有RCT和6项超过15例患者的病例研究的重要数据.评估的疗法包括奈莫珠单抗,阿瑞匹坦,局部用氢化可的松和吡美莫司治疗,沙利度胺,UVA光疗,普瑞巴林,还有纳曲酮.纳入的RCT和案例研究都有不同的方法,使得直接比较几乎是不可能的。
    结论:目前用于非特应性CNPG治疗的证据很少。一些关于新疗法的随机对照试验正在运行或正在进行中,希望提供新的,有效,以及有和没有特应性易感性的CNPG患者的针对性治疗可能性。
    BACKGROUND: Chronic nodular prurigo (CNPG) is a chronic, inflammatory skin disease, characterized by intense and debilitating pruritus. The pathophysiology is not fully understood, and the condition is difficult to treat with no targeted therapies. The aim of this systematic review was to review the evidence of therapies for non-atopic CNPG and conduct a meta-analysis of the results.
    CONCLUSIONS: We conducted a systematic review of the literature concerning effect of treatment for non-atopic CNPG. Due to few randomized controlled trials (RCTs) and case series, the literature was unfortunately too sparse to conduct a meta-analysis of the results. Instead, we thoroughly report important data from the three existing RCTs and 6 case studies with more than 15 patients. Evaluated therapies include nemolizumab, aprepitant, topical therapy with hydrocortisone and pimecrolimus, thalidomide, UVA phototherapy, pregabalin, and naltrexone. Included RCTs and case studies all had a heterogeneous methodology making direct comparison almost impossible.
    CONCLUSIONS: There is sparse evidence for the currently used therapies for non-atopic CNPG. Several RCTs on new therapies are running or in the pipeline, hopefully providing new, effective, and targeted treatment possibilities for CNPG patients both with and without an atopic predisposition.
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  • 文章类型: Journal Article
    背景:结节性痒疹(PN)是一种以强烈瘙痒为特征的慢性疾病,举起,结节性病变.因为目前没有美国食品和药物管理局批准的专门针对PN的疗法,管理是高度可变的,对治疗方案没有共识。
    目的:为帮助美国皮肤科医生诊断和有效治疗PN提供实践指导。
    方法:我们参加了一次圆桌讨论,从美国的角度就PN的诊断和治疗提出了共识建议。
    结果:PN的核心发现是公司的存在,结节性病变;瘙痒持续至少6周;和病史或体征,或者两者兼而有之,反复抓挠,采摘,或摩擦。诊断工作包括对系统进行全面审查,考虑到潜在的全身性疾病,和疾病严重程度的评估,包括疾病负担和瘙痒强度。应根据患者的临床表现选择治疗方法,合并症,和对先前治疗的反应,并应解决瘙痒的神经和免疫成分。
    结论:有关PN的数据来自轶事或小型临床试验,目前所有的治疗都是标签外使用的。
    结论:PN患者的有效治疗方法应基于临床判断,并根据患者的个人需求量身定制。
    BACKGROUND: Prurigo nodularis (PN) is a chronic disease characterized by intensely pruritic, raised, nodular lesions. Because there are currently no United States Food and Drug Administration-approved therapies specifically for PN, management is highly variable, and no consensus exists on treatment regimens.
    OBJECTIVE: To provide practical guidance to help United States dermatologists diagnose and effectively treat patients with PN.
    METHODS: We participated in a roundtable discussion to develop consensus recommendations on diagnosis and treatment of PN from a United States perspective.
    RESULTS: The core findings in PN are the presence of firm, nodular lesions; pruritus lasting at least 6 weeks; and a history or signs, or both, of repeated scratching, picking, or rubbing. The diagnostic workup involves a complete review of systems, considering potential systemic diseases, and assessment of disease severity, including disease burden and pruritus intensity. Treatment should be selected based on a patient\'s clinical presentation, comorbidities, and response to prior treatments and should address both neural and immunologic components of pruritus.
    CONCLUSIONS: Data on PN are from anecdotal or small clinical trials, and all treatments are currently used off-label.
    CONCLUSIONS: An effective treatment approach for patients with PN should be based on clinical judgment and tailored to the individual needs of the patient.
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