connective tissues disease

结缔组织病
  • 文章类型: Journal Article
    背景和目的指甲疾病包括广泛的条件,跨越先天性,发展,传染性,肿瘤,退化,皮肤病学,和系统性疾病。全面探讨其临床表现,发病率,和关联对于精确诊断和有效管理至关重要。方法这项观察性横断面研究在B.J.医学院和民用医院进行,从2017年7月至2019年6月,Ahmedabad涉及300例连续的指甲变化患者,报告了各种皮肤病和全身状况。纳入标准涉及两种性别和所有年龄组的患者,这些患者表现出与皮肤病和全身性疾病相关的指甲变化。数据收集需要全面的临床病史,全身和皮肤病学检查,使用皮肤镜(Dermlite3,10x)进行指甲评估,和补充测试。在MicrosoftExcel2007软件上进行分析。该研究得到了研究所伦理委员会的批准。结果300例患者中,女性的指甲受累率较高(57%),男女比例为1.3:1。受影响最大的年龄组是21-40岁,通常会受到6-10个指甲的影响。值得注意的是,家庭主妇的患病率较高。最常见的指甲状况是甲癣(24.33%),其次是牛皮癣指甲变化(20%)。较不频繁的指甲变化涉及湿疹(5.7%),甲沟炎(5%),药物诱导(4.3%),扁平苔藓(3.7%),外伤(3%),20个指甲营养不良(2.33%),达里尔病(2%),寻常型天疱疮(2%),斑秃(1.67%),中位海勒营养不良(1.33%),特应性皮炎(1%),大疱性表皮松解症(1%),球拍钉(1%),麻风病(1%),发痒糠疹(0.67%),白癜风(0.67%),二期梅毒(0.67%),先天性甲癣(0.67%),以及每个病例的全部白甲,甲下疣,Koenen肿瘤,和甲周纤维瘤(0.33%)。系统性自身免疫性结缔组织疾病(CTD)占9%;观察到的最常见的指甲发现是指甲褶皱红斑(48.1%),其次是指甲褶皱毛细血管扩张(44.4%)。在系统性硬化症(SS)中,最常见的发现是指甲褶皱毛细血管扩张症,在系统性红斑狼疮(SLE)中,最常见的是指甲褶皱红斑。在7例SS患者中发现了钉褶毛细血管镜上的硬皮病毛细血管模式,两名皮肌炎患者,只有一名SLE患者。在全身性疾病中观察到的指甲变化包括糖尿病和慢性肾衰竭患者的甲癣,缺血性心脏病和高血压的碎片出血,艾滋病毒中的纵向黑甲癣,缺铁性贫血中的甲沟炎和桔梗。其他系统性疾病,如Addison病和肾功能衰竭,还表现出各种指甲变化。结论除了它们的外观重要性之外,指甲具有重要的病理作用。精通指甲术语和分类是熟练评估的关键。了解正常和异常的指甲变异,以及他们的疾病关联,有利于诊断和量身定制的管理。钉子,经常被忽视但容易接近,作为了解患者一般健康状况的窗口,应该是彻底检查的一个组成部分。这项研究突出了指甲疾病的复杂临床全景,强调它们在皮肤病和系统环境中的重要作用。
    Background and objective Nail disorders encompass a wide spectrum of conditions, spanning congenital, developmental, infectious, neoplastic, degenerative, dermatological, and systemic diseases. A comprehensive exploration of their clinical manifestations, incidence, and associations is crucial for precise diagnosis and effective management. Methods This observational cross-sectional study conducted at B.J. Medical College and Civil Hospital, Ahmedabad involved 300 consecutive patients with nail changes from July 2017 to June 2019 reporting diverse dermatological and systemic conditions. The inclusion criteria involved patients of both genders and all age groups displaying nail changes associated with dermatological and systemic diseases. Data collection entailed a comprehensive clinical history, systemic and dermatological examinations, nail assessment using Dermoscope (DermLite 3, 10x), and supplementary tests. Analyses were performed on Microsoft Excel 2007 software. The study was approved by the Institute Ethics Committee. Results Among the 300 cases, females had a higher prevalence of nail involvement (57%), with a female-to-male ratio of 1.3:1. The most affected age group was 21-40 years, with 6-10 nails typically affected. Notably, housewives showed a higher prevalence. The most frequent nail condition was onychomycosis (24.33%) followed by psoriatic nail changes (20%). Less frequent nail changes involved eczema (5.7%), paronychia (5%), drug-induced (4.3%), lichen planus (3.7%), trauma-induced (3%), twenty nail dystrophy (2.33%), Darier\'s disease (2%), pemphigus vulgaris (2%), alopecia areata (1.67%), median Heller dystrophy (1.33%), atopic dermatitis (1%), epidermolysis bullosa (1%), racquet nail (1%), leprosy (1%), pityriasis rubra pilaris (0.67%), vitiligo (0.67%), secondary syphilis (0.67%), pachyonychia congenita (0.67%), as well as a case each of total leukonychia, subungual warts, Koenen tumor, and periungual fibroma(0.33%). Systemic autoimmune connective tissue disorders (CTD) accounted for 9%; the most common nail finding observed was nail fold erythema (48.1%) followed by nail fold telangiectasis (44.4%). In systemic sclerosis (SS), the most common finding was nail fold telangiectasia, and in systemic lupus erythematosus (SLE), the most common was nail fold erythema. Scleroderma capillary pattern on nail fold capillaroscopy was found in seven patients with SS, two patients with dermatomyositis, and only one patient with SLE. Nail changes observed in systemic diseases include onychomycosis in diabetes mellitus and chronic renal failure patients, splinter hemorrhages in ischemic heart disease and hypertension, longitudinal melanonychia in HIV, and koilonychia and platynychia in iron deficiency anemia. Other systemic diseases, such as Addison\'s disease and renal failure, also exhibited various nail changes. Conclusions Beyond their cosmetic importance, nails hold a vital pathologic role. Proficiency in nail terminology and classification is key for skillful evaluation. Understanding normal and abnormal nail variants, along with their disease associations, benefits diagnosis and tailored management. Nails, often overlooked but accessible, serve as a window into patients\' general health and should be an integral part of thorough examinations. This study highlights an intricate clinical panorama of nail disorders, highlighting their significant role in both dermatological and systemic contexts.
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  • 文章类型: Journal Article
    在大多数情况下,自身免疫性疾病的诊断对于临床医生来说是具有挑战性的,因为没有单一的特定实验室或组织学标记来诊断或排除病症的存在。这篇综述集中在目前对自身抗体检测在各种疾病中的作用的认识。比如系统性红斑狼疮,类风湿性关节炎,抗磷脂综合征,未分化结缔组织病,原发性胆汁性肝硬化和原发性硬化性胆管炎。同样,我们对间质性肺病患者自身抗体检测的预后和诊断价值进行了综述.对这些自身抗体在免疫调节和疾病发病机制中的分子作用的深入研究已经超出了它们与疾病表型的相关性,强调自身抗体靶向对疾病结局和病因的影响。
    Diagnosis of autoimmune diseases is in most cases challenging for clinicians as there is not a single specific laboratory or histological marker to diagnose or exclude the presence of the conditions. This review focused on the current knowledge of the role of autoantibodies\' testing in various diseases, such as systemic lupus erythematosus, rheumatoid arthritis, antiphospholipid syndrome, undifferentiated connective tissues disease, primary biliary cholangitis and primary sclerosing cholangitis. Similarly, the prognostic and diagnostic values of autoantibodies testing in patients with interstitial lung disease have been reviewed. In-depth research on the molecular action of these autoantibodies on immune regulation and diseases pathogenesis has been explored beyond their correlation with disease phenotypes, highlighting the impact of autoantibodies targeting on disease outcomes and etiopathogenesis.
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  • 文章类型: Journal Article
    背景:结缔组织疾病(CTD)是一组异质性疾病,具有某些临床特征和免疫调节功能紊乱。间质性肺病(ILD),也被称为弥漫性肺实质疾病,是与CTD相关的最严重的肺部并发症之一。白细胞介素9(IL-9),在CTD患者和CTD-ILD患者中研究了IL-4和干扰素γ(IFN-γ)-在自身免疫性疾病中具有重要作用的细胞因子。
    方法:招募61例住院未经治疗的CTD患者,20名健康志愿者作为对照.将61例CTD患者分为单纯CTD组和CTD-ILD组,酶联免疫吸附试验(ELISA)检测血浆蛋白IL-9、IL-4和IFN-γ水平。
    结果:结果表明,CTD-ILD患者和单纯CTD患者的血清IL-9水平明显高于健康对照组(均p<0.05),并且与单纯CTD患者相比,CTD-ILD患者的水平升高(p<0.05)。CTD-ILD患者的IL-4水平高于单纯CTD患者(p<0.05)和健康对照组(p<0.01)。此外,血清IL-9水平与IFN-γ水平呈负相关(r(2)=0.34,p=0.01),预测用力肺活量的估计百分比(FVC%)(r(2)=0.36,p=0.00)和预测扩散能力的估计百分比(DLCO%)(r(2)=0.27,p=0.04)与IL-4水平呈正相关(r(2)=0.31,p=0.01).
    结论:白细胞介素-9可能在CTD的发病中起重要作用,并可能参与CTD患者间质性肺损伤的进展。
    BACKGROUND: Connective tissues diseases (CTDs) are a heterogeneous group of disorders that share certain clinical characteristics and disturbed immunoregulation. Interstitial lung diseases (ILDs), also known as diffuse parenchymal lung diseases, are among the most serious pulmonary complications associated with CTDs. Interleukin 9 (IL-9), IL-4 and interferon γ (IFN-γ) - cytokines with important roles in autoimmune disease - were studied in CTD patients and CTD-ILD patients.
    METHODS: Sixty-one hospitalized untreated CTD patients were recruited, and 20 healthy volunteers were enrolled as controls. The 61 CTD patients were divided into a simple CTD group and a CTD-ILD group, and the plasma protein IL-9, IL-4 and IFN-γ levels were measured by enzyme-linked immunosorbent assay (ELISA).
    RESULTS: The results indicate that the serum IL-9 levels were significantly higher in CTD-ILD and simple CTD patients than they were in healthy controls (each p < 0.05) and that the levels were elevated in CTD-ILD patients compared with simple CTD patients (p < 0.05). The IL-4 levels were higher in CTD-ILD patients than they were in the simple CTD patients (p < 0.05) and healthy controls (p < 0.01). In addition, the serum IL-9 levels were negatively correlated with the level of IFN-γ (r (2) = 0.34, p = 0.01), the estimated percentage of predicted forced vital capacity (FVC%) (r (2) = 0.36, p = 0.00) and the estimated percentage of predicted diffusing capacity (DLCO%) (r (2) = 0.27, p = 0.04) and were positively correlated with the IL-4 level (r (2) = 0.31, p = 0.01).
    CONCLUSIONS: Interleukin-9 may play an important role in the pathogenesis of CTD and may contribute to the progression of interstitial lung injury in CTD patients.
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