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系统性红斑狼疮患者的临床特征与预后

Clinical Profile and Outcomes of Patients With Systemic Lupus Erythematosus.

作者信息

Bagare Prasad C, Borle Akshata, Baluni Priya, Ekbote Gayatri Gajanan, Sangale Shashikala

机构信息

Internal Medicine, Dr. DY Patil Medical College, Hospital and Research Centre, Pune, IND.

Rheumatology, Deenanath Mangeshkar Hospital and Research Centre, Pune, IND.

出版信息

Cureus. 2024 Sep 3;16(9):e68541. doi: 10.7759/cureus.68541. eCollection 2024 Sep.

Abstract

Background Systemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by relapsing-remitting immune system activation, affecting multiple organ systems. Despite significant advances in understanding SLE's pathogenesis, there remains a need for comprehensive clinical profiling at the time of diagnosis to improve early detection and management. This study addresses this gap by providing a detailed analysis of the clinical presentation, disease activity, and patient outcomes using the Systemic Lupus International Collaborating Clinics (SLICC) criteria and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) index. Methodology This cross-sectional observational study included 80 patients diagnosed with SLE using the 2012 SLICC criteria. Patients were recruited from the Rheumatology department and other wards of Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospital, Pune, India. All participants provided informed consent and institutional ethical approval was obtained. Data were collected through detailed clinical history, physical examinations, and standard tests such as chest X-rays, CBC, RFT, LFT, urine microscopy, creatine phosphokinase, ANA, AntiDsDNA, complement consumption, and Coombs' tests, with 2D echocardiography performed as needed. Follow-ups every three months over 1.5 years assessed disease activity using SLEDAI criteria. Patients aged 12 and above who met the SLICC criteria were included and those with other connective tissue disorders were excluded. Associations between clinical symptoms and organ involvement were analyzed using the chi-square test with a p-value of <0.05 considered significant. Results The study evaluated 80 patients with SLE, revealing a predominantly female cohort (80%) with a mean age of 29.4 years and a standard deviation of 8.3 years, skewed towards younger age groups. Clinical manifestations were diverse; the most common symptoms were (83.75%), oral ulcers (98.75%), and alopecia (95%). Anemia (66.25%) was the most prevalent abnormality, followed by albuminuria and renal abnormalities. Organ involvement was highest in the renal system (50%) and mucocutaneous features, with lower incidences in cardiac, gastrointestinal, and vascular systems. Gender-specific analyses indicated significant differences in SLE nephritis (p=0.048) and autoimmune hemolytic anemia (p=0.046). Autoantibody profiles showed high positivity for ANA (98.8%) and DsDNA (61.3%). Clinical outcomes demonstrated that 68.8% of patients achieved remission and 16.3% experienced organ damage. The SLEDAI scores significantly improved over time, with substantial reductions from baseline to nine months (p<0.001). Conclusion In conclusion, this study provides a detailed examination of SLE, revealing that it predominantly affects young adults and is characterized by diverse manifestations including mucocutaneous symptoms, significant renal involvement, and notable autoantibody profiles. The high prevalence of anti-nucleosome and anti-dsDNA antibodies underscores their diagnostic and prognostic value. Clinically, the findings highlight the necessity for early detection and targeted management of SLE, particularly in addressing renal and mucocutaneous symptoms. Future research should focus on longitudinal studies to track disease progression, explore genetic and environmental influences, and investigate regional variations to enhance treatment strategies and patient outcomes.

摘要

背景

系统性红斑狼疮(SLE)是一种复杂的自身免疫性疾病,其特征为免疫系统反复激活并缓解,可累及多个器官系统。尽管在理解SLE发病机制方面取得了重大进展,但在诊断时仍需要进行全面的临床分析,以改善早期检测和管理。本研究通过使用系统性红斑狼疮国际协作诊所(SLICC)标准和系统性红斑狼疮疾病活动指数(SLEDAI)指数,对临床表现、疾病活动度和患者预后进行详细分析,填补了这一空白。

方法

这项横断面观察性研究纳入了80例根据2012年SLICC标准诊断为SLE的患者。患者从印度浦那拜拉姆吉·杰吉博伊政府医学院和萨索恩综合医院的风湿病科及其他病房招募。所有参与者均提供了知情同意书,并获得了机构伦理批准。通过详细的临床病史、体格检查和标准检查(如胸部X光、血常规、肾功能、肝功能、尿液显微镜检查、肌酸磷酸激酶、抗核抗体、抗双链DNA、补体消耗和库姆斯试验)收集数据,并根据需要进行二维超声心动图检查。在1.5年的时间里,每三个月进行一次随访,使用SLEDAI标准评估疾病活动度。纳入年龄在12岁及以上且符合SLICC标准的患者,排除患有其他结缔组织疾病的患者。使用卡方检验分析临床症状与器官受累之间的关联,p值<0.05被认为具有统计学意义。

结果

该研究评估了80例SLE患者,结果显示以女性为主(80%),平均年龄为29.4岁,标准差为8.3岁,年龄分布偏向年轻人群体。临床表现多样;最常见的症状是(83.75%)、口腔溃疡(98.75%)和脱发(95%)。贫血(66.25%)是最常见的异常,其次是蛋白尿和肾脏异常。器官受累以肾脏系统(50%)和皮肤黏膜特征最为常见,心脏、胃肠道和血管系统的受累发生率较低。性别特异性分析显示,SLE肾炎(p = 0.048)和自身免疫性溶血性贫血(p = 0.046)存在显著差异。自身抗体谱显示抗核抗体(98.8%)和双链DNA(61.3%)的阳性率较高。临床结果表明,68.8%的患者实现了缓解,16.3%的患者出现了器官损伤。SLEDAI评分随时间显著改善,从基线到九个月有大幅下降(p < 0.001)。

结论

总之,本研究对SLE进行了详细检查,发现其主要影响年轻人,具有多种表现,包括皮肤黏膜症状、显著的肾脏受累和明显的自身抗体谱。抗核小体和抗双链DNA抗体的高患病率突出了它们的诊断和预后价值。临床上,这些发现强调了早期检测和针对性管理SLE的必要性,特别是在处理肾脏和皮肤黏膜症状方面。未来的研究应侧重于纵向研究,以跟踪疾病进展、探索遗传和环境影响,并调查区域差异,以改进治疗策略和患者预后。

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