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阿曼系统性红斑狼疮的发病谱:从儿童到成人。

Spectrum of systemic lupus erythematosus in Oman: from childhood to adulthood.

机构信息

College of Medicine, Sultan Qaboos University, Muscat, Oman.

Child Health Department, Sultan Qaboos University Hospital, Muscat, Oman.

出版信息

Rheumatol Int. 2018 Sep;38(9):1691-1698. doi: 10.1007/s00296-018-4032-2. Epub 2018 May 2.

DOI:10.1007/s00296-018-4032-2
PMID:29721697
Abstract

SLE is a disease that mainly affects women of childbearing age, however, a total of 15-20% of cases present in children. Although adult onset SLE (aSLE) and childhood onset SLE (cSLE) share the same diagnostic criteria, differences have been identified. The aim of this study is to compare the similarities and differences in between cSLE and aSLE in an Arab population from Oman. We evaluated 225 SLE patients, 139 adults and 86 children, who fulfilled the criteria for diagnosis. At disease onset, 99% of SLE cohort fulfilled the SLICC criteria; however the ACR 1997 criteria were fulfilled in 66% aSLE and 80% cSLE. The clinical features of SLE in cSLE showed higher frequency of renal (50 vs 19%; p < 0.001), musculoskeletal (67 vs 53%; p = 0.036) and pulmonary involvement (13 vs 2.9%, p = 0.005); while aSLE showed higher frequency of hematological (64 vs 49%; p = 0.25) and mucocutaneous (24 vs 10%; p = 0.13) involvement. The mean disease activity score at disease onset and during disease course was also higher in cSLE (13 vs 8.5; p < 0.0005) (16 vs 11.8; p < 0.0005), respectively. Differences in autoantibody profile were also noted in cSLE with higher positivity of anti-dsDNA and antiphospholipid antibody (94 vs 84%; p = 0.027) (53 vs 37%; p = 0.25), respectively. cSLE patients were more likely than aSLE to be treated with immunosuppressant such as cyclophosphamide (51 vs 22%; p < 0.001) and MMF (70 vs 54%; p = 0.019). Similarities and differences between aSLE and cSLE in a cohort from Oman of Arab ethnicity were identified. It appears that individual races and ethnicities may exhibit differences in disease susceptibility and manifestations.

摘要

SLE 是一种主要影响育龄妇女的疾病,但也有 15-20%的病例发生在儿童身上。尽管成人发病的系统性红斑狼疮 (aSLE) 和儿童发病的系统性红斑狼疮 (cSLE) 具有相同的诊断标准,但也存在差异。本研究的目的是比较阿拉伯国家阿曼人群中 cSLE 和 aSLE 之间的相似性和差异。我们评估了 225 例符合诊断标准的 SLE 患者,其中 139 例为成人,86 例为儿童。在疾病发作时,99%的SLE 患者符合 SLICC 标准;然而,66%的 aSLE 和 80%的 cSLE 符合 ACR 1997 标准。cSLE 患者的 SLE 临床特征显示,肾脏受累(50%比 19%;p<0.001)、肌肉骨骼(67%比 53%;p=0.036)和肺部受累(13%比 2.9%;p=0.005)的频率更高;而 aSLE 患者则表现为血液系统受累(64%比 49%;p=0.25)和黏膜皮肤受累(24%比 10%;p=0.13)的频率更高。cSLE 患者在疾病发作时和疾病过程中的平均疾病活动评分也更高(13 比 8.5;p<0.0005)(16 比 11.8;p<0.0005)。cSLE 患者的自身抗体谱也存在差异,抗 dsDNA 和抗磷脂抗体的阳性率更高(94%比 84%;p=0.027)(53%比 37%;p=0.25)。与 aSLE 患者相比,cSLE 患者更有可能接受环磷酰胺(51%比 22%;p<0.001)和 MMF(70%比 54%;p=0.019)等免疫抑制剂治疗。本研究在阿拉伯国家阿曼的队列中比较了 aSLE 和 cSLE 的相似性和差异。似乎不同种族和民族在疾病易感性和表现方面可能存在差异。

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