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儿科狼疮——与成人狼疮相比,其在临床表现、遗传学、对治疗的反应和损伤累积方面是否存在差异?

Pediatric lupus--are there differences in presentation, genetics, response to therapy, and damage accrual compared with adult lupus?

机构信息

Division of Rheumatology, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MC 4010, Cincinnati, OH 45229, USA.

出版信息

Rheum Dis Clin North Am. 2010 Feb;36(1):53-80, vii-viii. doi: 10.1016/j.rdc.2009.12.012.

Abstract

Some complement deficiencies predispose to systemic lupus erythematosus (SLE) early in life. Currently, there are no known unique physiologic or genetic pathways that can explain the variability in disease phenotypes. Children present with more acute illness and have more frequent renal, hematologic, and central nervous system involvement compared to adults with SLE. Almost all children require corticosteroids during the course of their disease; many are treated with immunosuppressive drugs. Mortality rates remain higher with pediatric SLE. Children and adolescents accrue more damage, especially in the renal, ocular and musculoskeletal organ systems. Conversely, cardiovascular mortality is more prevalent in adults with SLE.

摘要

某些补体缺陷可导致红斑狼疮(SLE)在生命早期发生。目前,尚无已知的独特生理或遗传途径可以解释疾病表型的可变性。与成人 SLE 相比,儿童表现出更严重的疾病,并且肾脏、血液系统和中枢神经系统受累更频繁。几乎所有儿童在疾病过程中都需要皮质类固醇;许多人接受免疫抑制剂治疗。儿科红斑狼疮的死亡率仍然较高。儿童和青少年会累积更多的损伤,尤其是在肾脏、眼部和肌肉骨骼系统。相反,心血管疾病的死亡率在成人 SLE 中更为常见。

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本文引用的文献

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Ethnic differences in pediatric systemic lupus erythematosus.儿童系统性红斑狼疮的种族差异。
J Rheumatol. 2009 Nov;36(11):2539-46. doi: 10.3899/jrheum.081141. Epub 2009 Oct 15.
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The socioeconomic burden of SLE.系统性红斑狼疮的社会经济负担。
Nat Rev Rheumatol. 2009 Jul;5(7):400-4. doi: 10.1038/nrrheum.2009.106. Epub 2009 Jun 9.
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Work disability in systemic lupus erythematosus.系统性红斑狼疮中的工作残疾
Arthritis Rheum. 2009 Mar 15;61(3):378-85. doi: 10.1002/art.24347.
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Disease recurrence in paediatric renal transplantation.小儿肾移植中的疾病复发
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