巴西单中心系列比较儿童,成人和晚发系统性红斑狼疮。

IF 1.9 4区 医学 Q3 RHEUMATOLOGY
Lupus Pub Date : 2025-10-01 Epub Date: 2025-08-08 DOI:10.1177/09612033251366394
Sean Hideo Shirata Lanças, Matheus Zanata Brufatto Furlan, Andrea de Almeida Peduti Batista, Sula Glaucia Lage Drumond, Taciana de Albuquerque Pedrosa Fernandes, Claudia Saad Magalhães, Cintia Zumstein Camargo
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SLE clinical profile, activity indices, remission, and damage comparison were carried out in childhood-onset (cSLE), adult-onset (aSLE) and late-onset (lSLE) patients from a single-centre series.MethodsA cross-sectional analysis reviewing the clinical profile of SLE cases seen between 2012 and 2022 and classified in 3 age-groups according to disease onset: cSLE (<18 years), aSLE (18 to 49 years) and lSLE (>=50 years), was performed. Disease activity status was assessed by PGA (0-3), SLEDAI-2K, SLE-DAS, LLDAS and DORIS criteria, and damage using SDI at the same visit were compared in those 3 age-groups.ResultsFour hundred and fifteen patients were included in the analysis: 289 (68%) aSLE, 79 (19%) cSLE, and 47 (11.3%) lSLE. Most common clinical manifestations were articular (75.2%), hematological (70.1%), cutaneous (67.9%), photosensitivity (59.3%), and renal (41.7%). 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引用次数: 0

摘要

系统性红斑狼疮(SLE)是一种异质性多系统自身免疫性疾病,具有不同的严重程度、自身抗体谱、治疗反应、复发过程和损害累积。发病年龄可能影响疾病发展轨迹和预后,主要脏器受累程度、疾病活动性和预后差异显著。对儿童期发病(cSLE)、成年期发病(aSLE)和晚发期(lSLE)患者的SLE临床特征、活动指数、缓解和损害进行了比较。方法对2012 - 2022年SLE病例的临床资料进行横断面分析,并根据发病情况分为3个年龄组:cSLE(=50岁)。采用PGA(0-3)、SLEDAI-2K、sledai - das、LLDAS和DORIS标准评估疾病活动状态,并比较3个年龄组在同一次就诊时使用SDI的损害情况。结果共纳入415例患者,其中aSLE 289例(68%),cSLE 79例(19%),lSLE 47例(11.3%)。最常见的临床表现为关节(75.2%)、血液学(70.1%)、皮肤(67.9%)、光敏(59.3%)和肾脏(41.7%)。SLE患者亚组的临床特征相似,除了lSLE患者肾炎和浆膜炎发生率较低,自身抗体和补体不足发生率较低,SLEDAI-2K和sledai - das评分较低,LLDAS和DORIS缓解率较高;和更高的伤害分数(SDI)。cSLE患者肾脏和神经系统受累的频率明显更高,病程更长,但疾病活动性、损害评分和缓解率与成人发病组相当。结论3个年龄组之间存在显著差异,青少年的肾脏和神经精神领域频率及疾病活动度评分高于其他年龄组。lle组肾炎和血清炎较少,疾病活动性较低,损伤评分较高。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Brazilian single-centre series comparision of childhood, adult and late onset Systemic lupus Erythematosus.

BackgroundSystemic Lupus Erythematosus (SLE) is a heterogeneous multisystem autoimmune disease, with variable severity, autoantibody profile, response to treatment, relapsing course and damage accrual. The age at disease onset may influence disease trajectory and prognosis, with remarkable differences of major organ involvement, disease activity, and prognosis. SLE clinical profile, activity indices, remission, and damage comparison were carried out in childhood-onset (cSLE), adult-onset (aSLE) and late-onset (lSLE) patients from a single-centre series.MethodsA cross-sectional analysis reviewing the clinical profile of SLE cases seen between 2012 and 2022 and classified in 3 age-groups according to disease onset: cSLE (<18 years), aSLE (18 to 49 years) and lSLE (>=50 years), was performed. Disease activity status was assessed by PGA (0-3), SLEDAI-2K, SLE-DAS, LLDAS and DORIS criteria, and damage using SDI at the same visit were compared in those 3 age-groups.ResultsFour hundred and fifteen patients were included in the analysis: 289 (68%) aSLE, 79 (19%) cSLE, and 47 (11.3%) lSLE. Most common clinical manifestations were articular (75.2%), hematological (70.1%), cutaneous (67.9%), photosensitivity (59.3%), and renal (41.7%). The clinical profile was similar among the subgroups of SLE patients, except for lSLE with lower frequency of nephritis and serositis, lower frequency of autoantibodies and hypocomplementenemia, lower SLEDAI-2K and SLE-DAS scores, and a higher frequency of LLDAS and DORIS remission; and a higher damage scores (SDI). Patients with cSLE had a significantly higher frequency of renal and neurological involvement, and a longer disease duration, but the disease activity, damage scores and remission rates were comparable to adult-onset group.ConclusionThe remarkable differences among the 3-age-groups were higher frequency of renal and neuropsychiatric domains in youngsters and disease activity scores compared to other age-groups. The lSLE group had less nephritis and serositis, lower disease activity and higher damage scores.

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来源期刊
Lupus
Lupus 医学-风湿病学
CiteScore
4.20
自引率
11.50%
发文量
225
审稿时长
1 months
期刊介绍: The only fully peer reviewed international journal devoted exclusively to lupus (and related disease) research. Lupus includes the most promising new clinical and laboratory-based studies from leading specialists in all lupus-related disciplines. Invaluable reading, with extended coverage, lupus-related disciplines include: Rheumatology, Dermatology, Immunology, Obstetrics, Psychiatry and Cardiovascular Research…
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