JIA

JIA
  • 文章类型: Case Reports
    背景:跛行是儿童时期常见的临床症状;不同的临床状况可能导致跛行,潜在病因的诊断往往是儿科医生面临的挑战。
    方法:我们描述了临床表现,放射学图片和其他原因导致儿童跛行的病程,通过7例病例系列和每种疾病的简要讨论。
    结论:虽然创伤是急性跛行的最常见原因,当没有创伤事件的历史并且跛行有慢性过程时,幼年特发性关节炎通常是最有可能的临床诊断。然而,其他一些罕见的情况下,如果JIA没有被证实或如果它呈现非典型的临床表现,应考虑在内。
    BACKGROUND: Limping is a common clinical symptom in childhood; different clinical conditions may lead to limping and the diagnosis of the underlying cause may often be a challenge for the pediatrician.
    METHODS: We describe the clinical manifestations, radiological pictures and disease course of other causes of limping in childhood, through a case series of seven cases and a brief discussion of each disease.
    CONCLUSIONS: although trauma is the most common cause of acute limping, when there is no history of traumatic events and the limping has a chronic course, Juvenile Idiopathic Arthritis is usually the most likely clinical diagnosis. However, other some rare conditions should be taken into account if JIA is not confirmed or if it presents with atypical clinical picture.
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  • 文章类型: Journal Article
    目前,关于COVID-19疾病(由SARS-CoV-2引起)对儿童风湿性疾病患者的影响的数据非常有限.为了评估可能的联系,我们测量了大流行期间幼年特发性关节炎(JIA)儿童和对照组的IgA和IgG抗SARS-CoV-2抗体水平,在引入抗COVID-19疫苗接种之前。我们评估了患者中PD-1抑制分子和炎症标志物的水平,并将这些结果与对SARS-CoV-2的血清学反应相关联。在JIA患者中,使用青少年关节炎疾病活动度评分71(JADAS71)量表评估疾病活动度.这项研究由96名儿童组成,65被诊断为JIA,用抗风湿药治疗,31名健康志愿者在JIA患者中,与对照组相比,IgA和IgG中的SARS-CoV-2抗体水平显着升高。我们还发现,与血清阴性者相比,SARS-CoV-2IgA或IgG抗体血清阳性的JIA患者和对照志愿者的血清PD-1水平明显更高。对SARS-CoV-2感染的体液免疫应答与JIA患者和健康儿童中PD-1表达的持续上调有关。检测到的疾病的临床意义需要进一步仔细观察。
    Currently, data regarding the impact of COVID-19 disease (caused by SARS-CoV-2) on patients with childhood rheumatic diseases are significantly limited. To assess the possible connection, we measured levels of IgA and IgG anti-SARS-CoV-2 antibodies in children with juvenile idiopathic arthritis (JIA) and a control group during the pandemic, prior to the introduction of anti-COVID-19 vaccination. We assessed levels of PD-1 suppressive molecule and inflammatory markers in patients and correlated those results with serological response to SARS-CoV-2. In JIA patients, the activity of the disease was assessed using the Juvenile Arthritis Disease Activity Score 71 (JADAS 71) scale. The study consisted of 96 children, 65 diagnosed with JIA, treated with antirheumatic drugs, and 31 healthy volunteers. In patients with JIA, significantly higher levels of SARS-CoV-2 antibodies in the IgA and IgG were demonstrated compared to the control group. We also found significantly higher serum PD-1 levels in JIA patients and control volunteers who were seropositive for SARS-CoV-2 IgA or IgG antibodies compared to those who were seronegative. The humoral immune response to SARS-CoV-2 infection is associated with the persistent upregulation of PD-1 expression in both JIA patients and healthy children. The clinical significance of the detected disorder requires further careful observation.
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  • 文章类型: Journal Article
    背景:治疗幼年特发性关节炎(JIA)相关葡萄膜炎需要长期使用皮质类固醇或免疫抑制剂,每个都有自己重要的副作用。初始治疗需要强化外用糖皮质激素,对于反应差或高危并发症如黄斑水肿的病例,采用免疫抑制剂的方法。迄今为止,只有很少的证据支持对这种复杂的亚组采取特定的方法.我们提出了第一个成功使用0.19mg醋酸氟轻松植入物的案例(ILUNEN®,AlimeraSciences,汉普郡,UK)作为一种新颖的装置,用于JIA并发葡萄膜炎的患者玻璃体内长期施用疾病修饰剂。
    方法:该回顾性病例报告描述了一名20岁女性,被诊断患有少关节JIA并伴有慢性葡萄膜炎和相关的囊样黄斑水肿(CMO)。考虑到包括患者不合规在内的因素,年龄,镜头状态,非类固醇反应,对短期玻璃体内类固醇治疗反应良好,0.19mg氟轻松玻璃体内植入剂被认为是一种合适的强化治疗选择.
    结果:在12个月的随访中,左眼(OS)显示视力从6/601(从0.98logMAR为0.42)(插入前)改善至6/15-1,中央视网膜厚度(CRT)从471μm减少199μm。右眼(OD),治疗10个月后,显示视力从6/24-1提高到6/7.5(从0.56LogMAR为0.10),治疗后6个月CRT减少327μm。
    结论:在这种情况下,0.19mg醋酸氟轻松植入物为JIA相关性葡萄膜炎和继发性CMO提供了安全有效的长期治疗.这可能为对短期皮质类固醇使用表现出良好反应的复杂病例提供替代方法。
    BACKGROUND: Treatment of juvenile idiopathic arthritis (JIA)-associated uveitis necessitates the use of long-term corticosteroids or immunosuppressive agents, each of which poses their own significant side effect profile. Initial treatment requires intensive topical glucocorticoids, with a step-up approach employing immunosuppressive agents for those cases with poor response or high-risk complications such as macular oedema. To date, there is minimal evidence to support a specific approach to such complicated subgroups. We present the first case to successfully employ the 0.19 mg fluocinolone acetonide implant (ILUVIEN®, Alimera Sciences, Hampshire, UK) as a novel device for prolonged intravitreal administration of disease-modifying agents for patients with JIA complicated by uveitis.
    METHODS: This retrospective case report describes a 20-year old woman diagnosed with oligoarticular JIA complicated by chronic uveitis and associated cystoid macular oedema (CMO). Considering factors including the patient\'s non-compliance, age, lens status, non-steroid response, and good response to short-term intravitreal steroid therapy, the 0.19 mg fluocinolone acetonide intravitreal implant was deemed an appropriate step-up treatment option.
    RESULTS: At 12-month follow-up, the left eye (OS) showed an improvement in visual acuity to 6/15 - 1 from 6/60 + 1 (0.42 from 0.98 logMAR) (pre-insertion) and a reduction in central retinal thickness (CRT) of 199 μm from 471 μm. The right eye (OD), treated 10 months later, showed an improvement in visual acuity to 6/7.5 from 6/24 - 1 (0.10 from 0.56 LogMAR) and a reduction in CRT of 327 μm 6 months after treatment.
    CONCLUSIONS: In this case, the 0.19 mg fluocinolone acetonide implant provided safe and effective long-term treatment of JIA-associated uveitis and secondary CMO. This potentially offers an alternative approach to complex cases that show good response to short-term corticosteroid use.
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  • 文章类型: Case Reports
    幼年特发性关节炎相关葡萄膜炎在日本人群中很少见。在这篇文章中,我们报告了一名儿童,他的黄斑水肿在接受阿达木单抗白内障手术后被控制了数年,并回顾了26例日本患者的文献。在这个案例报告中,一个4岁的男孩患上了带状角膜病,虹膜后粘连,和双眼复杂的白内障。由于两只眼睛的高眼压作为类固醇反应者,在另一家医院开出的口服泼尼松龙被停用。在5岁的时候,他开始每周口服甲氨蝶呤8mg治疗复发性双侧虹膜睫状体炎,然后在双眼接受晶状体切除术和核心玻璃体切除术.手术时取消了计划的人工晶状体植入,因为前玻璃体具有严重的炎性混浊,双眼均具有弥漫性视网膜水肿。由于术后5个月双眼持续性黄斑水肿,在6岁的时候,他开始每2周使用阿达木单抗注射液20mg.光学相干断层扫描显示的黄斑结构在2个月内恢复正常。在11岁的最后一次访问中,他的右眼最佳矫正视力为0.8,左眼为0.4,阿达木单抗每2周40mg和甲氨蝶呤每周8mg。总之,1例幼年特发性关节炎患儿,阿达木单抗可长期控制白内障手术后尽管口服甲氨蝶呤仍持续的黄斑水肿.在日本文学中,迄今仅报告了另外26例幼年特发性关节炎相关性葡萄膜炎.
    Juvenile idiopathic arthritis-associated uveitis is rare in the Japanese population. In this article, we report a child whose macular edema was controlled for years after cataract surgery with adalimumab, and reviewed 26 Japanese patients in the literature. In this case report, a 4-year-old boy developed band keratopathy, posterior iris synechiae, and complicated cataract in both eyes. Oral prednisolone prescribed at another hospital was discontinued due to high intraocular pressure in both eyes as a steroid responder. At the age of 5 years, he started oral methotrexate 8 mg weekly for recurrent bilateral iridocyclitis and then underwent lensectomy with core vitrectomy in both eyes. Planned intraocular lens implantation was cancelled at surgery because the anterior vitreous had severe inflammatory opacity with diffuse retinal edema in both eyes. Due to persistent macular edema in both eyes 5 months postoperatively, at the age of 6 years, he began to use adalimumab injection 20 mg every 2 weeks. The macular structure depicted by optical coherence tomography became normal in 2 months. At final visit at the age of 11 years, he had the best-corrected visual acuity of 0.8 in the right eye and 0.4 in the left eye, with adalimumab 40 mg every 2 weeks and methotrexate 8 mg weekly. In conclusion, macular edema persistent despite oral methotrexate after cataract surgery could be controlled for long term by adalimumab in a child with juvenile idiopathic arthritis. In the Japanese literature, only 26 additional cases with juvenile idiopathic arthritis-associated uveitis have been reported so far.
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  • 文章类型: Journal Article
    Pediatric rheumatologic disease occurs more frequently than several other chronic pediatric diseases but is often underrecognized. It is estimated that in the US, one in 250 children has some form of juvenile arthritis and 300,000 children have a form of rheumatologic disease. However, there are only approximately 400 practicing pediatric rheumatologists nationwide.
    Kern\'s six-step method was used to develop a pediatric rheumatology curriculum based on respondents\' perceived lack of training and comfort with four key areas: workup, musculoskeletal exam, laboratory interpretation, and referral to rheumatology. These cases were developed for second-year pediatric and second- and third-year internal medicine-pediatric residents rotating with the service. The curriculum was composed of four 30-minute case discussions as well as an observed musculoskeletal exam session.
    In 2017, weekly case study sessions reached 34 trainees. Survey results from these trainees are representative of our overall results and reveal that learners felt the content of the cases helped increase comfort with compiling pertinent history and information of symptoms consistent with autoimmune disease, recognizing physical exam findings of autoimmune disease, ordering and interpreting laboratory studies in children with concerns for autoimmune disease, and referring to pediatric rheumatology.
    This case-based curriculum exposed residents to presentations of the more common autoimmune diseases encountered in the pediatric population. The curriculum helps fill a gap in pediatric training through increased exposure to this subset of chronic diseases and expands physical examination skills not typically taught in general pediatrics.
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