哈萨克斯坦共和国系统性红斑狼疮患者的临床表现、病程、结局和健康相关生活质量特点

B. Issayeva, E. Aseeva, M. Saparbayeva, S. Issayeva, M. Kulshymanova, S. Kaiyrgali, A. S. Amanzholov, M. Bizhanova, M. Kalykova, S. Solovyev, N. Akhtaeva
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摘要

目的:评价哈萨克斯坦共和国系统性红斑狼疮患者的临床表现、病程、结局和与健康相关的生活质量特点。患者和方法。本研究纳入102例系统性红斑狼疮(SLE)患者,根据SLICC(2012)进行可靠诊断。用SLEDAI 2K指数评估疾病活动性,用SLICC/ACR评估器官损伤(IOD)(2000)。采用SPSS 13软件(IBM Corp., USA)进行统计处理。具有参数分布的变量以М±SD表示,非参数-为中位数(Me) [25;第75个百分位)。结果和讨论。该队列以女性患者(98%)、亚洲人(83.33%)、年轻患者(33.85±10.58岁)为主,病程5年[2];[9]年,活动度高(30.8%)和极高(39.2%)(SLEDAI-2K - 17.64±8.80分)。18.6%的患者在青春期发病,其特点是病程不佳。临床表现:皮肤损害(急性活动性和慢性形式)(98%),关节(79.4%),非瘢痕性脱发(75.5%),神经精神障碍(49%),粘膜(46.1%),血液(54.9%)和免疫障碍(100%)。IOD:低(20.6%),中(59.8%),高(9.8%),零(9.8%),93.1%的患者存在导致预后不良的危险因素。SLE患者的健康相关生活质量评估(HRQOL)在所有量表上均显示显著下降。纠正治疗方案,考虑不良后果因素(FRNI),包括加强治疗,纳入基因工程生物药物(GEBP)。SLE是哈萨克斯坦的一种具有社会意义的疾病,在10年(2009-2018年)中发病率很高(101%)。SLE患者以年轻人、女性为主。病程最长可达5年,SLE诊断的验证延迟。器官损伤在发病时就已经存在,93.1%的患者存在FRNI,这表明病程的严重性,需要早期诊断并积极参与包括GEBD在内的发病治疗。
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Features of clinical manifestations, course, outcomes and health related quality of life in patients with systemic lupus erythematosus in the Republic of Kazakhstan
Objective – to evaluate the features of clinical manifestations, course, outcomes and quality of life related to health in patients with systemic lupus erythematosus in the Republic of Kazakhstan.Patients and methods. The study included 102 patients with systemic lupus erythematosus (SLE) with a reliable diagnosis according to SLICC (2012). Disease activity was assessed by the SLEDAI 2K index, organ damage (IOD) by SLICC/ACR (2000). Statistical processing was carried out using SPSS 13 software (IBM Corp., USA). Variables with a parametric distribution are presented as М±SD, nonparametric – as a median (Me) [25th; 75th percentile].Results and discussion. The cohort was dominated by female patients (98%), Asians (83.33%), young patients (33.85±10.58 years) with a disease duration of 5 [2; 9] years with high (30.8%) and very high (39.2%) degree of activity (SLEDAI-2K – 17.64±8.80 points). The debut of the disease was in 18.6% of patients in adolescence, it was characterized by an unfavorable course. Clinical manifestations of the disease: skin lesions (acute active and chronic forms) (98%), joints (79.4%), non-scarring alopecia (75.5%), neuropsychiatric disorders (49%), mucous membranes (46.1%), hematological (54.9%) and immunological disorders (100%). IOD: low – in 20.6%, medium – in 59.8%, high – in 9.8% of patients, 0 – in 9.8%, Risk factors for poor outcome were in 93.1% of patients. Assessment of health-related quality of life (HRQOL) in SLE patients showed a significant decrease on all scales. Correction of the treatment program, taking into account the factors of adverse outcome (FRNI), consisted in strengthening therapy with the inclusion of genetically engineered biological drugs (GEBP).Conclusion. SLE is a socially significant disease in Kazakhstan with a high incidence rate (101%) over 10 years (2009–2018). The cohort of SLE patients is dominated by young people, females. The duration of the disease is up to 5 years with a delayed verification of the diagnosis of SLE. Organ damage is already in the onset of the disease and the presence of FRNI of the disease in 93.1% of patients, which indicates the severity of the course, which requires early diagnosis and active involvement of pathogenetic treatment, including GEBD. 
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