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对300例土耳其系统性红斑狼疮患者的临床、免疫学特征、损伤及生存率的分析。

Analysis of clinical, immunological characteristics, damage, and survival in 300 Turkish systemic lupus erythematosus patients.

作者信息

Tekeoglu Senem, Temiz Karadag Duygu, Ozdemir Isik Ozlem, Yazici Ayten, Cefle Ayse

机构信息

Department of Rheumatology, Internal Medicine, Halic University, Istanbul, Turkey.

Department of Rheumatology, Internal Medicine, Kocaeli University, Kocaeli, Turkey.

出版信息

Lupus. 2024 Mar;33(3):298-311. doi: 10.1177/09612033241228174. Epub 2024 Jan 23.

Abstract

OBJECTIVE

This retrospective study aimed to conduct a comprehensive analysis of Turkish Systemic Lupus Erythematosus (SLE) patients of Caucasian ethnicity, focusing on their clinical, immunological, and therapeutic characteristics, damage accural and mortality.

PATIENTS AND METHODS

We carried out a retrospective assessment of 300 SLE patients diagnosed between 2001 and 2017 at Kocaeli University Rheumatology Clinic. Demographic data, clinical manifestations, immunological profiles, treatment approaches, disease-related damage, and survival information were collected.

RESULTS

The study population had a significant female predominance (89%) with a mean age of disease onset of 35.4 (SD:13.3) years. Hematological (72.6%) and mucocutaneous (72%) manifestations were the most common clinical findings, followed by arthritis (66.3%). Females had higher frequency of photosensitivity (p = 0.019), malar rash (p < 0.001), and alopecia (p = 0.014). Anti-dsDNA antibodies were detected in 61.6% of patients, while 57% of patients had hypocomplementemia. Secondary antiphospholipid syndrome was observed in 15.3% of patients; the most common manifestations included deep venous thrombosis (32.6%) and cerebrovascular accidents (30.4%). Lupus Nephritis (LN) affected 40.3% of the cohort. The most common pathologic finding was Class IV LN (30.5%). Eventually, 13 (4.3%) patients developed chronic kidney disease (CKD) and 4 had renal replacement therapies. Patients with LN had higher usage of pulse steroids, azathioprin, mycophenolate mofetil, cyclophosphamide, and rituximab (p < 0.001 for each).  In the juvenile-onset group (n = 31, 10.3%), an increased occurrence of malar rash (p = 0.009), nephritis (p = 0.034), hypocomplementemia (p = 0.001), positive anti-dsDNA (p = 0.007), anti-Sm (p = 0.046), anti-rib-P (p = 0.014) antibodies were observed. At least one damage parameter was observed in 32.6% patients with musculoskeletal manifestations being the most common. Thirteen patients were diagnosed with various malignancies, with cervical cancer being the most common (4 cases). The total 5 and 10-year survival rates were 92.5% and 86.7%, respectively. However, patients with CKD, had lower survival rates; 75% at 3 years and 60% at 15 years. Regression analysis demonstrated an association of CKD and  history of infections with decreased survival (p = 0.02, each).

CONCLUSION

Ethnicity and geography influence the clinical diversity of SLE. Recognizing these disparities is crucial for tailoring patient care. Future inception cohort studies in Turkish SLE patients are necessary to address the limitations of retrospective research.

摘要

目的

本回顾性研究旨在对高加索族裔的土耳其系统性红斑狼疮(SLE)患者进行全面分析,重点关注其临床、免疫学和治疗特征、损伤累积情况及死亡率。

患者与方法

我们对2001年至2017年在科贾埃利大学风湿病诊所确诊的300例SLE患者进行了回顾性评估。收集了人口统计学数据、临床表现、免疫谱、治疗方法、疾病相关损伤和生存信息。

结果

研究人群中女性占比显著(89%),疾病发病的平均年龄为35.4(标准差:13.3)岁。血液学(72.6%)和黏膜皮肤(72%)表现是最常见的临床发现,其次是关节炎(66.3%)。女性的光敏性(p = 0.019)、蝶形红斑(p < 0.001)和脱发(p = 0.014)发生率更高。61.6%的患者检测到抗双链DNA抗体,而57%的患者有补体低下。15.3%的患者观察到继发性抗磷脂综合征;最常见的表现包括深静脉血栓形成(32.6%)和脑血管意外(30.4%)。狼疮性肾炎(LN)影响了该队列的40.3%。最常见的病理发现是IV级LN(30.5%)。最终,13例(4.3%)患者发展为慢性肾脏病(CKD),4例接受了肾脏替代治疗。LN患者使用脉冲类固醇、硫唑嘌呤、霉酚酸酯、环磷酰胺和利妥昔单抗的频率更高(每项p < 0.001)。在青少年发病组(n = 31,10.3%)中,观察到蝶形红斑(p = 0.009)、肾炎(p = 0.034)、补体低下(p = 0.001)、抗双链DNA阳性(p = 0.007)、抗Sm(p = 0.046)、抗核糖体P(p = 0.014)抗体的发生率增加。32.6%的患者观察到至少一个损伤参数出现,其中肌肉骨骼表现最为常见。13例患者被诊断患有各种恶性肿瘤,其中宫颈癌最为常见(4例)。总的5年和10年生存率分别为92.5%和86.7%。然而,CKD患者的生存率较低;3年时为75%,15年时为

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