immune-mediated inflammatory disorder

  • 文章类型: Case Reports
    成人发作的斯蒂尔病(AOSD)是一种令人困惑的疾病,临床表现多样,对医疗保健专业人员构成重大诊断挑战。本病例报告深入探讨了临床轨迹,诊断挑战,治疗策略,以及一名67岁女性AOSD患者所经历的结果。本报告主张将AOSD视为存在全身性炎症症状的患者的潜在诊断。尤其是在其他条件被排除的情况下。它强调了AOSD的复杂性和跨学科合作的重要性,密切监测,和个性化的治疗策略,以优化患者的治疗效果。
    Adult-onset Still\'s disease (AOSD) stands as a perplexing condition with diverse clinical manifestations, posing significant diagnostic challenges for healthcare professionals. This case report delves into the clinical trajectory, diagnostic challenges, treatment strategies, and outcomes experienced by a 67-year-old female with AOSD. This report advocates for considering AOSD as a potential diagnosis in patients presenting with systemic inflammatory symptoms, especially when other conditions have been ruled out. It highlights the complexity of AOSD and the importance of interdisciplinary collaboration, close monitoring, and personalized treatment strategies to optimize patient outcomes.
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  • 文章类型: Case Reports
    噬血细胞性淋巴组织细胞增生症(HLH)是一种威胁生命的综合征,涉及过度的免疫激活。它可以是原发性(家族性)或继发性(由感染引发,恶性肿瘤,或风湿病)。这是一个以前健康的43岁非洲裔美国妇女的案例,她出现了发烧和混乱。患者最终被诊断为肺曲霉病,对抗真菌治疗反应良好。她符合HLH-2004治疗噬血细胞性淋巴组织细胞增生症的诊断标准。她还符合2019年系统性红斑狼疮(SLE)的分类标准,没有SLE的经典体征和症状。HLH管理包括支持性管理,潜在疾病的治疗,和免疫抑制治疗。依托泊苷和地塞米松是HLH的常用治疗方法;然而,潜在的活动性感染可能会限制治疗选择。在我们的案例中,患者接受类固醇和羟氯喹治疗.她的病情逐渐好转,没有出现并发症。根据我们的文献综述,我们遇到6例继发于曲霉病的HLH病例,平均年龄约47岁.HLH的诊断通常由于非特异性表现而延迟。早期识别和治疗对提高生存率至关重要。
    Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome involving excessive immune activation. It can be primary (familial) or secondary (triggered by infection, malignancy, or rheumatological disease). This is a case of a previously healthy 43-year-old African American woman who presented with fever and confusion. The patient was eventually diagnosed with pulmonary aspergillosis and responded well to antifungal therapy. She met the diagnostic criteria of HLH-2004 trial for hemophagocytic lymphohistiocytosis. She also fulfilled the 2019 classification criteria for systemic lupus erythematosus (SLE) without the classical signs and symptoms of SLE. HLH management includes supportive management, treatment of underlying condition, and immunosuppressive treatment. Etoposide and dexamethasone are commonly used treatments for HLH; however, underlying active infection can limit the treatment options. In our case, the patient was treated with steroids and hydroxychloroquine. Her condition gradually improved and she recovered without complications. Based on our literature review, we encountered six cases of HLH secondary to Aspergillosis with a mean age of approximately 47 years. The diagnosis of HLH is often delayed because of nonspecific presentation. Early identification and treatment are crucial to improve the survival rate.
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  • 文章类型: Journal Article
    背景SARS-CoV-2(COVID-19)是冠状病毒家族的正链核糖核酸(RNA)病毒,这导致了最严重的流行病之一,全球确诊病例超过1400万例。估计类风湿性关节炎(RA)在0.5-1%的美国人口中流行。到目前为止,几乎没有证据表明COVID-19感染及其导致RA患者死亡率或住院时间增加的倾向.为了对这一文学做出贡献,本研究将评估COVID-19与RA患者死亡率和住院时间增加相关的程度,同时考虑这些患者的合并症.方法我们的回顾性研究包括14,180例患者(年龄>18,中位数58,范围18-90),这些患者从1月1日起检测出COVID-19阳性或被认为感染了COVID-19,2020年7月31日,2020年。根据RA和COVID-19感染的诊断将患者与没有RA的患者进行分组。被诊断为系统性红斑狼疮(SLE)的患者,慢性阻塞性肺疾病,排除高血压。协变量包括年龄,体重指数(BMI),种族,性别,最大C反应蛋白值,最大D-二聚体值,合并糖尿病。结果指标是住院时间(LOS),住院死亡率,重症监护病房(ICU)入院,ICULOS,机械通气,机械通气时间,出院到临终关怀医院.Logistic回归模型用于估计住院死亡率的概率,入住ICU,放置在机械通气上,出院到临终关怀,当比较RA和COVID-19感染的患者与COVID-19感染的非RA患者时,院内死亡率与家庭抗炎使用相关。结果14180例患者中(男性57.1%,女性42.9%),159名患者(1.1%),诊断为RA。RA和医院LOS之间没有显著关联,入住ICU,ICULOS,机械通气的LOS,或在感染COVID-19的人中出院到临终关怀。然而,在感染COVID-19的患者中,RA与较高的死亡率(OR:1.65;95%CI:1.07-2.53;p=0.02)和机械通气(OR:1.82;95%CI:1.22-2.71;p<0.01)相关。结论这项研究表明,RA和COVID-19患者院内死亡率和机械通气的可能性显着增加。虽然在大流行的情况下很难意识到,有必要在全国范围内开展大型研究,以提高我们对诊断为RA的患者中COVID-19感染的认识.
    Background SARS-CoV-2 (COVID-19) is a positive-stranded ribonucleic acid (RNA) virus of the coronavirus family, which has resulted in one of the most serious pandemics, with more than 14 million cases confirmed globally. Rheumatoid arthritis (RA) is estimated to be prevalent in 0.5-1% of the U.S. population. So far, there has been little evidence of COVID-19 infection and its propensity to result in increased mortality or length of hospital stay in patients with RA. To contribute to this body of literature, this study will assess the degree to which COVID-19 is associated with increased mortality and length of hospital stay in patients with RA while also taking into account these patients\' comorbidities. Methods Our retrospective study included 14,180 patients (age >18, median 58, range 18-90) who tested positive for COVID-19 or were assumed to have COVID-19 infection from January 1st, 2020, through July 31st, 2020. Patients were grouped based on the diagnosis of RA and COVID-19 infection versus those without RA. Patients who were diagnosed with systemic lupus erythematosus (SLE), chronic obstructive pulmonary disease, and hypertension were excluded. Covariates included age, body mass index (BMI), race, sex, maximum C-reactive protein value, maximum D-dimer value, and comorbid diabetes mellitus. Outcome measures were length of hospital stay (LOS), in-hospital mortality, intensive care unit (ICU) admission, ICU LOS, mechanical ventilation, time on mechanical ventilation, and discharge to hospice. The logistic regression model was used to estimate the probability of in-hospital mortality, ICU admission, placement on mechanical ventilation, discharge to hospice, and in-hospital mortality related to home anti-inflammatory use when comparing patients with RA and COVID-19 infection to COVID-19 infected patients without RA. Results Of the total 14,180 patients (males 57.1%, females 42.9%), 159 patients (1.1%), had a diagnosis of RA. There was no significant association between RA and hospital LOS, ICU admission, ICU LOS, LOS on mechanical ventilation, or discharge to hospice among those infected with COVID-19. Yet, RA was associated with higher mortality (OR: 1.65; 95% CI: 1.07-2.53; p=0.02) and placement on mechanical ventilation (OR: 1.82; 95% CI: 1.22-2.71; p<0.01) amidst patients infected with COVID-19. Conclusion This study suggests that patients with RA and COVID-19 have a significantly increased likelihood of in-hospital mortality and placement on mechanical ventilation. While challenging to realize in a pandemic situation, large studies nationwide are necessary to improve our understanding of COVID-19 infection in patients diagnosed with RA.
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  • 文章类型: Case Reports
    我们报告了一名15岁的儿科患者的非典型病例,该患者被诊断为肺炎支原体相关的急性横贯性脊髓炎(ATM)。患者没有通常与支原体感染相关的前驱或肺部症状。然而,患者表现为急性双侧下肢瘫痪,感觉异常,在T11及以下水平的感觉下降,肠和膀胱功能障碍,和血小板减少症.脊髓的磁共振成像显示从T10到延髓圆锥末端的横贯性脊髓炎。尽管使用阿奇霉素进行治疗,但患者的临床改善缓慢,大剂量静脉注射甲基强的松龙,静脉注射免疫球蛋白,和血浆置换.本报告呼吁注意早期识别支原体作为ATM的潜在原因的重要性,以及延迟检测和治疗的潜在后果:更严重的神经系统并发症,住院时间延长,和不利的临床结果。
    We report an atypical case of a 15-year-old pediatric patient diagnosed with Mycoplasma pneumoniae associated acute transverse myelitis (ATM). The patient had no prodromal or pulmonary symptoms that are commonly associated with mycoplasma infection. Yet, the patient exhibited acute bilateral lower extremity paralysis, paresthesia, decreased sensation at the level of T11 and below, bowel and bladder dysfunction, and thrombocytopenia. Magnetic resonance imaging of the spinal cord revealed transverse myelitis from T10 to the end of the conus medullaris. The patient showed only slow clinical improvement despite therapy consisting of azithromycin, high-dose intravenous methylprednisolone, intravenous immunoglobulin, and plasmapheresis. This report calls attention to the importance of early identification of mycoplasma as an underlying cause of ATM and the potential consequences of delayed detection and treatment: more severe neurologic complications, prolonged hospitalization, and unfavorable clinical outcomes.
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