ANA

ANA
  • 文章类型: Journal Article
    牛皮癣是一种慢性炎症性疾病,有时需要生物制剂的治疗干预。在使用肿瘤坏死因子(TNF)抑制剂治疗期间产生自身抗体是一种公认的现象,然而,在银屑病患者中,与抗磷脂综合征(APS)相关的自身抗体的产生尚未得到全面评估.这项研究是为了评估接受不同生物制剂治疗的银屑病患者中APS相关自身抗体的患病率,并研究自身抗体产生与临床或血清学参数之间的潜在关联。接受生物制剂治疗的银屑病患者参加了这项研究,并根据所管理的生物制品的类型进行分类,TNF,白介素(IL)-17或IL-23抑制剂。收集临床和血清学数据,并结合APS自身抗体数据进行分析。与IL-17和IL-23抑制剂相比,TNF抑制剂与较高频率的APS自身抗体相关。值得注意的是,在接受TNF抑制剂治疗的患者中,APS自身抗体的存在与并发关节炎和开始治疗时更高的疾病严重程度相关.银屑病面积和严重程度指数评分升高以及抗核抗体滴度高于×320是APS自身抗体产生的预测因子。尽管自身抗体率较高,这些患者没有APS的临床症状.这项研究提供了第一个全面的证据,表明银屑病患者中与TNF抑制剂治疗相关的APS自身抗体频率增加。观察到的APS自身抗体阳性与TNF抑制剂治疗或临床参数之间的关联表明,在牛皮癣的发病机理中,自身免疫与炎症之间存在潜在的免疫调节相互作用。
    Psoriasis is a chronic inflammatory disease that sometimes necessitates therapeutic intervention with biologics. Autoantibody production during treatment with tumor necrosis factor (TNF) inhibitors is a recognized phenomenon, however, the production of autoantibodies associated with antiphospholipid syndrome (APS) has not been comprehensively evaluated in patients with psoriasis. This study was conducted to assess the prevalence of APS-associated autoantibodies in patients with psoriasis treated with different biologics and to investigate the potential associations between autoantibody production and clinical or serological parameters. Patients with psoriasis undergoing biologics treatments were enrolled in this study, and were categorized based on the type of biologics administered, TNF, interleukin (IL)-17, or IL-23 inhibitors. Clinical and serological data were collected and analyzed in conjunction with data on APS autoantibodies. TNF inhibitors were associated with a higher frequency of APS autoantibodies compared to IL-17 and IL-23 inhibitors. Notably, the presence of APS autoantibodies correlated with concurrent arthritis and higher disease severity at treatment initiation in patients treated with TNF inhibitors. Elevated Psoriasis Area and Severity Index scores and anti-nuclear antibody titers higher than × 320 were predictors of APS autoantibody production. Despite the higher autoantibody rates, clinical symptoms of APS were absent in these patients. This study provides the first comprehensive evidence of an increased frequency of APS autoantibodies associated with TNF inhibitor treatment in patients with psoriasis. The observed association between APS autoantibody positivity and TNF inhibitor treatment or clinical parameters suggests a potential immunomodulatory interplay between autoimmunity and inflammation in the pathogenesis of psoriasis.
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  • 文章类型: Journal Article
    免疫系统对SARS-CoV-2的扩增反应可能导致自身抗体的产生,但它们对疾病严重程度和结局的具体影响尚不清楚.这项研究旨在评估住院的COVID-19患者是否基于ANA的存在而面临更差的预后,即使没有自身免疫性疾病。我们做了一个回顾,单中心,观察性队列研究,于2020年4月至2021年3月在福贾(意大利)的“PoliclinicoRiuniti”医院招募638名COVID-19患者住院。ANA检测阳性的COVID-19患者的30天生存率显着降低(64.4%vs.83.0%),住院期间发生严重呼吸系统并发症的可能性高于ANA筛查阴性的患者(35.4%vs.17.0%)(p<0.001)。通过计算HALP评分(血红蛋白-白蛋白-淋巴细胞-血小板)来确定不良预后与ANA状态之间的关联,与ANA阴性患者相比,ANA检测阳性的COVID-19患者的这一比例较低(108.1±7.4vs.218.6±11.2AU;p<0.011)。详细来说,低HALP的COVID-19患者的30天生存率较低(99.1%与83.6%与高55.2%,中等,和低HALP,分别;p<0.001),与高和中HALP患者相比,不良呼吸事件的发生率更高(13.1%vs.35.2%与64.6%为高,中等,和低HALP,分别为;p<0.001)。总之,COVID-19患者的ANA阳性似乎与更具侵袭性的疾病表型相关,生存率降低。此外,我们认为HALP评分可作为评估COVID-19患者预后的一个有价值的参数.
    The immune system\'s amplified response to SARS-CoV-2 may lead to the production of autoantibodies, but their specific impact on disease severity and outcome remains unclear. This study aims to assess if hospitalized COVID-19 patients face a worse prognosis based on ANA presence, even without autoimmune diseases. We performed a retrospective, single-center, observational cohort study, enrolling 638 COVID-19 patients hospitalized from April 2020 to March 2021 at Hospital \"Policlinico Riuniti\" of Foggia (Italy). COVID-19 patients with a positive ANA test exhibited a significantly lower 30-day survival rate (64.4% vs. 83.0%) and a higher likelihood of severe respiratory complications during hospitalization than those with negative ANA screening (35.4% vs. 17.0%) (p < 0.001). The association between poor prognosis and ANA status was identified by calculating the HALP score (Hemoglobin-Albumin-Lymphocyte-Platelet), which was lower in COVID-19 patients with a positive ANA test compared to ANA-negative patients (108.1 ± 7.4 vs. 218.6 ± 11.2 AU; p < 0.011). In detail, COVID-19 patients with a low HALP showed a lower 30-day survival rate (99.1% vs. 83.6% vs. 55.2% for high, medium, and low HALP, respectively; p < 0.001) and a higher incidence of adverse respiratory events compared to those with high and medium HALP (13.1% vs. 35.2% vs. 64.6% for high, medium, and low HALP, respectively; p < 0.001). In summary, ANA positivity in COVID-19 patients appears to be linked to a more aggressive disease phenotype with a reduced survival rate. Furthermore, we propose that the HALP score could serve as a valuable parameter to assess prognosis for COVID-19 patients.
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  • 文章类型: Journal Article
    背景:护理专业,连同其各自的专业组织,超越了时间的沧桑。这包括,但不限于,职业的演变和非洲裔美国护士融入护理组织和领导角色。
    目的:美国护士协会(ANA)的三位非洲裔美国总统被邀请参加关于他们的领导和总统任期的口述历史。
    方法:访谈是视觉/音频记录的,数字录音,并转录。
    结论:口述历史集中在他们成为ANA总裁的旅程上,当总统的经历,超越他们的总统任期,以及各自对总统职位的见解。
    BACKGROUND: The nursing profession, along with its respective professional organizations, has transcended through the vicissitudes of time. This includes, but is not limited to, the evolution of the profession and integration of African American nurses into nursing organizations and leadership roles.
    OBJECTIVE: The three past African American presidents of the American Nurses Association (ANA) were invited to participate in an oral history about their leadership and presidencies.
    METHODS: The interviews were visual/audio-recorded, digitally taped, and transcribed.
    CONCLUSIONS: The oral histories centered on their journeys to becoming the president of the ANA, experiences being the president, leading beyond their presidency, and respective insights about their presidency.
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  • 文章类型: Journal Article
    背景:亚洲自身免疫性肝炎(AIH)的特征大多仍难以捉摸。
    方法:在台湾三级治疗癌症患者中进行了一项肝活检证实的AIH患者的队列研究。
    结果:从1999年到2022年,在13,766例接受肝活检的患者中,纳入150例AIH患者。男女比例为2.26。在基线,平均年龄为51.09岁,平均丙氨酸转氨酶水平为494.11U/L,和17(11.3%)有肝硬化。除一名患者外,所有患者均为1型AIH。女性年龄较大,基线肝硬化发生率高于男性。肝硬化的23年累积发病率,肝细胞癌(HCC),死亡率/肝移植,自身免疫性疾病和肝外肿瘤占64.2%,13.3%,23.4%,30.7%和21.2%,分别。1年,2年,3年,5年,10年和20年后的免疫抑制治疗复发率为60%,78.2%,81.8%,89.1%,94.5%和100%,分别。基线关联如下:碱性磷酸酶(Alk-p)水平与免疫抑制治疗后爆发[危险比(HR):1.003;95%CIHR:1.000-1.005];患有HCC的年龄(1.072;1.010-1.138)和全因癌症(1.041;1.005-1.079);肝硬化伴死亡率/肝移植(11.933;1.984-71.00A,与1.肝硬化(1.001;1.000-1.002),和自身免疫性疾病(1.001;1.000-1.002)。
    结论:在一个病毒性肝炎流行的亚洲国家,AIH患者的女性对男性和基线肝硬化率低于预期,而超过60%的患者最终发展为肝硬化。高的治疗后复发率需要谨慎监测,特别是对于基线Alk-p水平高的患者。基线年龄,肝硬化状态和ANA滴度对预后至关重要.
    BACKGROUND: The characteristics of autoimmune hepatitis (AIH) in Asia mostly remain elusive.
    METHODS: A cohort study of liver biopsy-proven AIH patients was conducted in a tertiary care cancer of Taiwan.
    RESULTS: From 1999 to 2022, of 13,766 patients who underwent liver biopsy, 150 patients with AIH were enrolled. The female-to-male ratio was 2.26. At baseline, the mean age was 51.09 years, mean alanine aminotransferase level was 494.11 U/L, and 17 (11.3%) had cirrhosis. All except one patient had AIH type 1. The females were older and had higher baseline cirrhosis rates than did the males. The 23-year cumulative incidences of cirrhosis, hepatocellular carcinoma (HCC), mortality/liver transplantation, autoimmune diseases and extrahepatic cancer were 64.2%, 13.3%, 23.4%, 30.7% and 21.2%, respectively. The 1-year, 2-year, 3-year, 5-year, 10-year and 20-year postimmunosuppressive therapy relapse rates were 60%, 78.2%, 81.8%, 89.1%, 94.5% and 100%, respectively. Baseline associations were as follows: alkaline phosphatase (Alk-p) levels with postimmunosuppressive therapy flare [hazard ratio (HR): 1.003; 95% CI HR: 1.000-1.005]; age with HCC (1.072; 1.010-1.138) and all-cause cancer (1.041;1.005-1.079); cirrhosis with mortality/liver transplantation (11.933;1.984-71.787); and antinuclear antibody (ANA) titers with mortality/liver transplantation (1.001;1.000-1.003), cirrhosis (1.001;1.000-1.002), and autoimmune diseases (1.001; 1.000-1.002).
    CONCLUSIONS: In an Asian country endemic for viral hepatitis, the female-to-male and baseline cirrhosis rates of AIH patients were lower than expected, while over 60% of the patients eventually developed cirrhosis. The high posttherapy relapse rate warrants cautious monitoring, particularly for patients with high baseline Alk-p levels. Baseline age, cirrhosis status and ANA titers are crucial for outcomes.
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  • 文章类型: Journal Article
    背景:精神病综合征在某些患者中可能有自身免疫介导的原因。因此,这项回顾性研究旨在探讨风湿病标志物在精神病发展中的作用。
    方法:总共,224名接受“风湿病实验室筛查”的精神病综合征患者(包括C反应蛋白[CRP],免疫固定,补充因素,类风湿因子[RF],抗磷脂抗体[APAs],抗中性粒细胞胞浆抗体[ANCAs],和抗核抗体[ANA])进行了分析。进一步的诊断检查包括神经元抗体和脑脊液(CSF)的调查,以及大脑的脑电图(EEG)和磁共振成像(MRI)。在所有患者中使用人上皮瘤-2(Hep2)细胞的血清常规进行ANA测试,一部分患者(N=73)也接受了基于组织的血清和CSF检测。描述性收集了自身免疫性精神病综合征的病例数,对ANA阳性和阴性患者进行了详细比较。
    结果:9%的患者CRP升高,免疫固定鉴定出8%的改变,补体因子C3下降14%,射频升高1%,APA升高了7%,ANCA并不明显积极,19%的ANAs阳性(可提取的核抗原[ENA]分化导致14例患者的阳性发现)。从使用基于组织的测定法额外调查的73例患者样本中,某种ANA有26个阳性结果(36%),总的来说,使用这两种方法,54名患者(24%)被认为是ANAs阳性。神经精神病学评估显示,七名患者(3%)可能存在自身免疫性精神病综合征,两名患者(1%)可能存在自身免疫性精神病综合征。ANA阳性患者接受抗抑郁药治疗的频率更高(p=0.040),并且躯体合并症的数量更高(p<0.001)。此外,与ANA阴性患者相比,在ANA阳性患者中发现(慢性)炎性MRI病变(p=0.008)和局灶性萎缩(p=0.012)的频率更高。
    结论:风湿病筛查导致4%的人怀疑可能或可能的自身免疫性精神病综合征。ANA与MRI病理相关。因此,在极少数情况下,风湿病过程可能导致精神病综合征的发展。
    BACKGROUND: Psychotic syndromes can have autoimmune-mediated causes in some patients. Thus, this retrospective work aims to investigate the role of rheumatological markers in the development of psychosis.
    METHODS: In total, 224 patients with psychotic syndromes receiving a \"rheumatological laboratory screening\" (including C-reactive protein [CRP], immunofixation, complement factors, rheumatoid factor [RF], antiphospholipid antibodies [APAs], antineutrophil cytoplasmic antibodies [ANCAs], and antinuclear antibodies [ANAs]) were analyzed. A further diagnostic work-up included investigations of neuronal antibodies and cerebrospinal fluid (CSF), as well as electroencephalography (EEG) and magnetic resonance imaging (MRI) of the brain. ANA testing was routinely performed in all patients using serum on human epithelioma-2 (Hep2) cells, and a subset of patients (N = 73) also underwent tissue-based assays from serum and CSF. The number of cases with autoimmune psychotic syndromes was descriptively collected, and ANA-positive and -negative patients were compared in detail.
    RESULTS: CRP was elevated in 9 % of patients, immunofixation identified alterations in 8 %, complement factor C3 was decreased in 14 %, RF was elevated in 1 %, APAs were elevated in 7 %, ANCAs were not clearly positive, and ANAs were positive in 19 % (extractable nuclear antigen [ENA] differentiation resulted in positive findings in 14 patients). From the 73 patient samples additionally investigated using tissue-based assays, there were 26 positive results for some kind of ANA (36 %), and overall using both methods, 54 patients (24 %) were considered positive for ANAs. A neuropsychiatric evaluation revealed a possible autoimmune psychotic syndrome in seven patients (3 %) and a probable autoimmune psychotic syndrome in two patients (1 %). ANA-positive patients were more frequently treated with antidepressants (p = 0.040) and had a higher number of somatic comorbidities (p < 0.001). In addition, (chronic) inflammatory MRI lesions (p = 0.008) and focal atrophies (p = 0.012) were found more frequently in ANA-positive than ANA-negative patients.
    CONCLUSIONS: Rheumatological screening led to suspicion of a possible or probable autoimmune psychotic syndrome in 4%. ANAs were associated with MRI pathologies. Therefore, rheumatological processes may contribute to the development of psychotic syndromes in rare cases.
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  • 文章类型: Journal Article
    大创伤导致的细胞碎片可能会压倒清除剂机制并导致自身免疫反应。我们分析了人类严重明确的创伤是否会在侮辱后的几个月内引起短暂自身免疫的实验室体征。我们在一项前瞻性队列研究中纳入了50例股骨转子骨折患者,这些患者接受了髓内钉接骨术,并在3-4天进行了随访。6周,术后12周和12个月。通过标准技术,我们评估了总免疫球蛋白的水平,抗核抗体(ANA),抗心磷脂抗体,抗dsDNA抗体和抗C1q抗体,以及针对巨细胞病毒(CMV)的抗体作为对照。在基线和前两个术后样本中确定血液白细胞差异和淋巴细胞亚群。患者的平均年龄达到80.1岁,23人(46%)完成所有访问。血清总IgG浓度,IgM和IgA在所有随访时间点增加。ANA荧光强度单位在术后12周和12个月增加(p<0.0001),但ANA阳性患者的比例没有变化(35%).在所有随访中,抗C1q值略有增加,但不是总IgG的比例。抗dsDNA在所有患者中保持阴性,和抗心磷脂IgG/IgM抗体没有变化。抗CMVIgG抗体在所有随访中显著增加,与总IgG的比例没有变化。流式细胞术显示术后3-4天B细胞比例增加。总之,老年患者的主要肌肉骨骼创伤引起免疫球蛋白产生的普遍非特异性增加,而没有增强的全身性自身免疫的实验室体征。
    Cellular debris resulting from large trauma might overwhelm the scavenger mechanisms and lead to autoimmune reactions. We analysed whether a major well-defined trauma in humans induces laboratory signs of transient autoimmunity in the months after the insult. We included 50 patients with pertrochanteric femur fracture undergoing intramedullary nail osteosynthesis in a prospective cohort study and followed them at 3-4 days, 6 weeks, 12 weeks and 12 months postoperatively. By standard techniques, we assessed levels of total immunoglobulins, anti-nuclear antibodies (ANA), anti-cardiolipin antibodies, anti-dsDNA antibodies and anti-C1q antibodies, as well as antibodies against cytomegalovirus (CMV) as a control. Blood leukocyte differential and lymphocyte subpopulations were determined at baseline and in the first two postoperative samples. The mean age of the patients reached 80.1 years, and 23 (46%) completed all visits. Serum concentrations of total IgG, IgM and IgA increased at all follow-up time points. The ANA fluorescence light intensity units increased at 12 weeks and 12 months postoperatively (p < 0.0001), but the proportion of ANA-positive patients did not change (35%). The values of anti-C1q mildly increased at all follow-up visits, but not the ratio to total IgG. Anti-dsDNA remained negative in all patients, and anti-cardiolipin IgG/IgM antibodies did not change. Anti-CMV IgG antibodies increased significantly at all follow-up visits, without change in the ratio to total IgG. Flow cytometry showed an increased proportion of B-cells 3-4 days postoperatively. In conclusion, major musculoskeletal trauma in elderly patients induces a generalized non-specific increase in immunoglobulin production without laboratory signs for enhanced systemic autoimmunity.
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  • 文章类型: Journal Article
    将近一半的系统性红斑狼疮(SLE)患者出现肺部受累。该研究评估了SLE患者肺部受累的程度,并确定了人群中的相关因素。
    这项回顾性队列研究在沙特阿拉伯南部地区的Aseer医院和KhamisMyshat医院进行。该研究从2016年1月1日至2023年6月3日。患者纳入标准包括根据美国风湿病学会标准接受明确诊断和分类的个体。而18岁以下的患者和混合性结缔组织疾病的患者被排除在外。
    共包括247名参与者。41.7%(n=103),41岁及以上,95.1%(n=235)为女性。约10.10%患有糖尿病,17.00%患有高血压和甲状腺功能减退症。狼疮性肾炎占15.40%。胸部受累报告占21.9%,以胸膜炎的形式(6.10%),胸腔积液(4.00%),和狼疮肺炎(4.00%),间质性肺病(4.00%),肺栓塞(3.60%),肺出血(2.80%)。SLE报告的呼吸道症状是;呼吸困难,咳嗽,胸痛的患病率约为18.0%。心悸的发生率相对较高,为13.80%。同时,咯血(咳嗽)的患病率较低,为1.20%,据报道,发烧率为2.80%。患有慢性肾脏疾病和高血压与肺部受累显着相关;(χ2=3.308,p=0.027)和(χ2=7.782,Fisher的p=0.002)。抗磷脂抗体的血清阳性,反CCP,Antids-DNA与肺部受累显着相关(χ2=3.239,=p=0.049),(χ2=4.621,费希尔p=0.023),(χ2=8.248,p=0.010)。
    研究发现,21.9%的SLE患者出现胸部受累,有不同程度的肺部症状。慢性肾病等因素,高血压,抗磷脂抗体,反CCP阳性,和抗dsDNA的血清阳性被发现与肺受累显著相关,有助于我们对SLE的理解。
    UNASSIGNED: Nearly half of the Systemic lupus erythematosus (SLE) patients develop lung involvement. The study assessed the extent of pulmonary involvement among SLE patients and to identify the associated factors in the population.
    UNASSIGNED: This retrospective cohort study was conducted at Aseer Hospital and Khamis Myshat Hospital in the Southern region of Saudi Arabia. The study spanned from January 1, 2016, to June 3, 2023. Patient inclusion criteria encompassed individuals who received a definitive diagnosis and classification as per American College of Rheumatology criteria, while patients under 18 years of age and those with mixed connective tissue diseases were exclude.
    UNASSIGNED: A total of 247 participants were included. 41.7% (n=103) aged 41 years and older, 95.1% (n = 235) were females. Around 10.10% had diabetes mellitus and 17.00% had hypertension and hypothyroidism. Lupus Nephritis was in 15.40%. Chest involvement was reported in 21.9%, in the form of pleuritis (6.10%), pleural effusion (4.00%), and lupus pneumonitis (4.00%), interstitial lung disease (4.00%), pulmonary embolism (3.60%) of individuals, and pulmonary hemorrhage (2.80%). The respiratory symptoms reported by SLE were; dyspnea, cough, and chest pain each having a prevalence of around 18.0%. Palpitations have a relatively high occurrence at 13.80%. Meanwhile, hemoptysis (blood coughing) has a lower prevalence of 1.20%, and fever is reported at 2.80%. Having chronic kidney disease and hypertension were significantly associated with having pulmonary involvement; (χ2=3.308, p=0.027) and (χ2=7.782, Fisher\'s p=0.002) respectively. The seropositivity for antiphospholipid Abs, anti-CCP, and antids-DNA were significantly associated with pulmonary involvement (χ2=3.239, =p=0.049), (χ2=4.621, Fisher\'s p=0.023), and (χ2=8.248, p=0.010) respectively.
    UNASSIGNED: The study found that 21.9% of SLE patients experience chest involvement, with varying degrees of pulmonary symptoms. Factors such as chronic kidney disease, hypertension, antiphospholipid antibodies, Anti-CCP positivity, and seropositivity for Anti-dsDNA were found to be significant associations with lung involvement, contributing to our understanding of SLE.
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  • 文章类型: Journal Article
    背景:为了评估幼年特发性关节炎(JIA)患者的长期结局和预后因素,表现为少关节炎,他们接受IAC作为他们疾病的第一种治疗方法。
    方法:我们在卢布尔雅那大学儿童医院进行了回顾性研究,斯洛文尼亚,从2015年1月到2023年5月,JIA儿童,临床上表现为少关节炎,接受关节内皮质类固醇注射(IAC)作为初始治疗。收集患者和治疗数据,根据以后的治疗需要,将结果分为三组:不需要治疗,只需要额外的IAC和全身治疗.最后一组根据bDMARD的要求进一步划分。Log-rank(Mantel-Cox)生存分析比较了不同结果组。
    结果:我们纳入了109例JIA患者,表现为少关节炎(63%为女性),首先接受IAC治疗的人。IAC的平均年龄是8.0岁,4.3年随访。值得注意的是,38.5%的患者在IAC后不需要额外的治疗,而15.5%只需要额外的IAC。全身治疗,主要是甲氨蝶呤(MTX),对45.9%的患者来说是必要的,平均在IAC后7.8个月内开始。在IAC后的平均2.2年内,22%的人开始了生物治疗。注射关节的数量与生物制剂的需求相关。在最后一次随访中,88.9%患有非活动性疾病。ANA阳性(P=0.049,卡方3.89)和HLAB27抗原存在(P=0.050,卡方3.85)与全身治疗的需要相关。一个8岁以上儿童的亚组,与其他患者相比,ANA和HLAB27阴性患者所需的全身治疗(25.8%)和生物治疗(9.6%)明显较少(p=0.050,卡方3.77)。
    结论:需要IAC的少关节JIA患儿中几乎40%没有进展为慢性疾病。年龄更小,ANA阳性,HLAB27的存在是全身治疗的预测因素,而注射关节的数量预测了未来对生物治疗的需求。
    BACKGROUND: To evaluate long-term outcomes and prognostic factors in patients with juvenile idiopathic arthritis (JIA), presenting as oligoarthritis, who received IAC as the first treatment for their disease.
    METHODS: We conducted retrospective study at the University Children\'s Hospital Ljubljana, Slovenia, from January 2015 to May 2023 in children with JIA, clinically presenting as oligoarthritis receiving intra-articular corticosteroid injection (IAC) as the initial treatment. Patient and treatment data were collected, and the outcomes were categorized into three groups based on the later need for therapy: no therapy needed, only additional IAC needed and systemic therapy needed. The last group was further divided based on the requirement of bDMARD. Log-rank (Mantel-Cox) survival analyses compared different outcome groups.
    RESULTS: We included 109 patients with JIA, presenting as oligoarthritis (63% female), who were first treated with IAC. The mean age at IAC was 8.0 years, with a 4.3-year follow-up. Notably, 38.5% of patients did not require additional therapy post-IAC, whereas 15.5% required only additional IAC. Systemic therapy, mainly methotrexate (MTX), was necessary for 45.9% of patients, initiated in average 7.8 months post-IAC. Biologic therapy was initiated in 22% in average 2.2 years post-IAC. Number of injected joints correlated with the need for biologics. At the last follow-up, 88.9% had inactive disease. ANA positivity (P = 0.049, chi square 3.89) and HLA B27 antigen presence (P = 0.050, chi square 3.85) were associated with the need for systemic therapy. A subgroup of children older than 8 years, ANA and HLA B27 negative required significantly less systemic (25.8%) and biologic therapy (9.6%) compared to other patients (p = 0.050, chi square 3.77).
    CONCLUSIONS: Almost 40% of children with oligoarticular JIA requiring IAC did not progress to chronic disease. Younger age, ANA positivity, and HLA B27 presence were predictive factors for systemic therapy, while the number of injected joints predicted the future need for biologic therapy.
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  • 文章类型: Journal Article
    系统性红斑狼疮(SLE)是一种复杂的疾病,临床表现隐匿。在多达一半的案例中,SLE发病的特点是临床和血清学表现,虽然具体,不足以满足分类标准。这个条件,叫做不完整SLE,可能与明确和可分类的SLE一样具有挑战性,需要根据临床表现的严重程度进行治疗。此外,早期诊断和治疗干预可以积极影响疾病的结果,包括缓解率和损害累积。诊断后,大多数患者的病程是复发缓解。缓解时间和糖皮质激素累积暴露是影响预后的最重要因素。因此,及时识别SLE临床模式可能有助于针对疾病过程进行治疗干预。迟发性SLE是罕见的,但更常与诊断延迟和合并症的发生率相关。包括干燥综合征.本文对SLE的病程进行综述,为早期诊断提供可行的策略,系统性红斑狼疮可能的临床模式的概述,以及与不同发病年龄SLE组相关的临床变异。
    Systemic lupus erythematosus (SLE) is a complex disease with an insidious clinical presentation. In up to half of the cases, SLE onset is characterized by clinical and serological manifestations that, although specific, are insufficient to fulfill the classification criteria. This condition, called incomplete SLE, could be as challenging as the definite and classifiable SLE and requires to be treated according to the severity of clinical manifestations. In addition, an early SLE diagnosis and therapeutic intervention can positively influence the disease outcome, including remission rate and damage accrual. After diagnosis, the disease course is relapsing-remitting for most patients. Time in remission and cumulative glucocorticoid exposure are the most important factors for prognosis. Therefore, timely identification of SLE clinical patterns may help tailor the therapeutic intervention to the disease course. Late-onset SLE is rare but more often associated with delayed diagnosis and a higher incidence of comorbidities, including Sjogren\'s syndrome. This review focuses on the SLE disease course, providing actionable strategies for early diagnosis, an overview of the possible clinical patterns of SLE, and the clinical variation associated with the different age-at-onset SLE groups.
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  • 文章类型: Journal Article
    背景:小儿免疫性血小板减少症(ITP)可能先于系统性自身免疫性疾病。在青少年ITP患者中,系统性红斑狼疮(SLE)的常规筛查可通过检测抗核抗体(ANA)滴度进行.羟氯喹(HCQ)是SLE患者安全有效的免疫调节药物,但很少用于ITP。我们分析了HCQ治疗儿童SLE相关ITP患者的血小板计数反应和安全性。
    方法:一项回顾性研究,包括患有ITP和明确或不完全SLE的儿科患者,他们在2010-2021年间接受了HCQ治疗。SLE定义为通过免疫荧光测量的ANA滴度≥1:160,根据2019年EULAR/ACR2019分类标准≥10分,而不完全SLE患者得分<10。血小板计数的完全反应(CR)定义为血小板计数>100×109/L;部分反应(PR)为血小板计数30-100×109/L,超过≥两倍的基线计数。
    结果:在纳入的17名患者中(中位年龄15.5岁;IQR3.6),15人(88.2%)为女性,13有明确的SLE,和四个有不完整的SLE。HCQ在ITP诊断后的中位17个月开始,中位血小板计数为38×109/L(IQR28)。在8周的时候,8例(47.1%)患者有反应,其中6人(35.3%)达到CR。一年后,总体反应为82.4%,其余血小板计数稳定的患者不需要额外的ITP治疗。在42个月的中位随访中维持了反应。没有注意到对HCQ的不利影响。
    结论:患有SLE相关ITP的儿科患者可能从HCQ治疗中获益。
    BACKGROUND: Pediatric immune thrombocytopenia (ITP) may precede systemic autoimmune disorders. In adolescent patients with ITP, routine screening for systemic lupus erythematosus (SLE) may be performed by testing for antinuclear antibody (ANA) titer. Hydroxychloroquine (HCQ) is a safe and effective immunomodulatory drug in patients with SLE but rarely used in ITP. We analyzed the platelet count response and safety of HCQ in treating pediatric patients with SLE-related ITP.
    METHODS: A retrospective study including pediatric patients with ITP and definite or incomplete SLE, who were treated with HCQ during 2010-2021. SLE was defined by ANA titer ≥ 1:160 as measured by immunofluorescence and ≥10 points according to the 2019 EULAR/ACR 2019 classification criteria, while patients with incomplete SLE achieved a score < 10. Complete response (CR) of the platelet count was defined as platelet count > 100 × 109/L; partial response (PR) as platelet count 30-100 × 109/L and exceeding ≥ twice baseline counts.
    RESULTS: Of the 17 patients included (median age 15.5 years; IQR 3.6), 15 (88.2%) were female, 13 had definite SLE, and four had incomplete SLE. HCQ was initiated at a median of 17 months after ITP diagnosis with a median platelet count of 38 × 109/L (IQR 28). At 8 weeks, 8 (47.1%) patients responded, including 6 (35.3%) achieving CR. After one year, the overall response was 82.4%, with the remaining patients having stable platelet counts requiring no additional ITP therapy. The response was maintained at a median follow-up of 42 months. No adverse effects to HCQ were noted.
    CONCLUSIONS: Pediatric patients with SLE-related ITP may benefit from treatment with HCQ.
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