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土耳其两家三级中心联合队列中青少年起病型和成人起病型系统性红斑狼疮患者的疾病特征、器官损害及生存率比较

Comparison of Disease Characteristics, Organ Damage, and Survival in Patients with Juvenile-onset and Adult-onset Systemic Lupus Erythematosus in a Combined Cohort from 2 Tertiary Centers in Turkey.

作者信息

Artim-Esen Bahar, Şahin Sezgin, Çene Erhan, Şahinkaya Yasemin, Barut Kenan, Adrovic Amra, Özlük Yasemin, Kılıçaslan Işın, Omma Ahmet, Gül Ahmet, Öcal Lale, Kasapçopur Özgür, İnanç Murat

机构信息

From the Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; Department of Statistics, Faculty of Arts and Sciences, Yıldız Technical University; Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.

B. Artim-Esen, MD, Associate Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; S. Şahin, MD, Fellow, Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; E. Çene, Research Assistant, Department of Statistics, Faculty of Arts and Sciences, Yıldız Technical University; Y. Şahinkaya*, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; K. Barut, MD, Fellow, Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; A. Adrovic, MD, Fellow, Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine; Y. Özlük, PhD, Associate professor, Department of Pathology, Istanbul Faculty of Medicine, Istanbul University, I. Kılıçaslan, PhD, Professor, Department of Pathology, Istanbul Faculty of Medicine, Istanbul University; A. Omma**, MD, Fellow, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; A. Gül, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine; L. Öcal, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University; Ö. Kasapçopur, MD, Professor, Division of Rheumatology, Department of Pediatrics, Cerrahpaşa Faculty of Medicine, Istanbul University; M. İnanç, MD, Professor, Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University.

出版信息

J Rheumatol. 2017 May;44(5):619-625. doi: 10.3899/jrheum.160340. Epub 2017 Mar 15.

Abstract

OBJECTIVE

Age at onset has been shown to affect the clinical course and outcome of systemic lupus erythematosus (SLE). Herein, we aimed to define the differences in clinical characteristics, organ damage, and survival between patients with juvenile-onset (jSLE) and adult-onset SLE (aSLE).

METHODS

For the study, 719 patients (76.9%) with aSLE and 216 (23.1%) with jSLE were examined. Comparisons between the groups were made for demographic characteristics, clinical features, auto-antibody profiles, damage, and survival rates.

RESULTS

These results were significantly more frequent in jSLE: photosensitivity, malar rash, oral ulcers, renal involvement, neuropsychiatric (NP) manifestations, and autoimmune hemolytic anemia (AIHA). Of the autoantibodies, a higher frequency of anti-dsDNA and anticardiolipin IgG and IgM were observed in the jSLE group. A significant proportion of patients with aSLE had anti-Sm positivity and pleuritis. The proportion of patients with jSLE who developed organ damage was comparable to that of patients with aSLE (53% vs 47%) and the mean damage scores were similar in both groups. Renal damage was significantly more frequent in jSLE while musculoskeletal and cardiovascular system damage and diabetes mellitus were more prominent in aSLE. Comparison of survival rates of the 2 groups did not reveal any significant differences.

CONCLUSION

We report a higher frequency in the jSLE group of renal involvement, cutaneous symptoms, oral ulcers, NP manifestations, AIHA, and anti-dsDNA positivity. A significant proportion of patients in the jSLE group had damage, most prominently in the renal domain. Our findings might support different genetic/environmental backgrounds for these 2 subgroups.

摘要

目的

发病年龄已被证明会影响系统性红斑狼疮(SLE)的临床病程和结局。在此,我们旨在明确青少年起病的SLE(jSLE)和成人起病的SLE(aSLE)患者在临床特征、器官损害及生存率方面的差异。

方法

本研究纳入了719例aSLE患者(76.9%)和216例jSLE患者(23.1%)。对两组患者的人口统计学特征、临床特征、自身抗体谱、损害情况及生存率进行了比较。

结果

jSLE患者中以下情况更为常见:光敏性、颊部皮疹、口腔溃疡、肾脏受累、神经精神(NP)表现及自身免疫性溶血性贫血(AIHA)。在自身抗体方面,jSLE组中抗双链DNA、抗心磷脂IgG和IgM的出现频率更高。相当一部分aSLE患者抗Sm阳性且有胸膜炎。jSLE患者发生器官损害的比例与aSLE患者相当(53%对47%),两组的平均损害评分相似。jSLE患者肾脏损害更为常见,而肌肉骨骼和心血管系统损害以及糖尿病在aSLE患者中更为突出。两组生存率比较未发现显著差异。

结论

我们报告jSLE组肾脏受累、皮肤症状、口腔溃疡、NP表现、AIHA及抗双链DNA阳性的频率更高。jSLE组中相当一部分患者存在损害,最显著的是在肾脏方面。我们的研究结果可能支持这两个亚组存在不同的遗传/环境背景。

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