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儿童与成人免疫球蛋白A血管炎(过敏性紫癜)的比较:来自土耳其的一项单中心研究

Comparing immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children and adults: a single-centre study from Turkey.

作者信息

Batu E D, Sarı A, Erden A, Sönmez H E, Armağan B, Kalyoncu U, Karadağ Ö, Bilginer Y, Akdoğan A, Kiraz S, Özen S

机构信息

a Division of Rheumatology, Department of Pediatrics , Hacettepe University Faculty of Medicine , Ankara , Turkey.

b Division of Rheumatology, Department of Internal Medicine , Hacettepe University Faculty of Medicine , Ankara , Turkey.

出版信息

Scand J Rheumatol. 2018 Nov;47(6):481-486. doi: 10.1080/03009742.2018.1448111. Epub 2018 Jun 18.

Abstract

OBJECTIVE

Immunoglobulin A vasculitis/Henoch-Schönlein purpura (IgAV/HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It is the most common primary systemic vasculitis of childhood and is much less common in adults. Our aim was to investigate the differences and similarities between adult and paediatric patients with IgAV/HSP.

METHOD

We retrospectively evaluated the medical records of 35 adult and 159 paediatric (˂ 18 years old) patients with a clinical diagnosis of IgAV/HSP who were seen at the Departments of Rheumatology and Pediatric Rheumatology, Hacettepe University, Ankara, Turkey. The paediatric and adult patients were classified with IgAV/HSP according to the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively.

RESULTS

Upper respiratory tract infection was a common predisposing factor for both adults (34.3%) and children (21.4%). Creatinine and C-reactive protein were higher; and skin biopsy, hypertension, renal involvement, haematuria, proteinuria, and renal insufficiency at diagnosis were more frequent in adults than in children. Thrombocyte count was higher in children than in adults. Follow-up without treatment and complete recovery were more frequent in children, while persistent haematuria, chronic renal failure, relapse, and the use of corticosteroids/azathioprine were more frequent in adults. The only independent predictive factor for relapse was persistent haematuria.

CONCLUSION

Various clinical and laboratory characteristics differ between children and adults with IgAV/HSP. Overall, IgAV/HSP has a self-limiting course in children but represents a more severe form of disease in adults, with more severe renal involvement. Persistent haematuria is a predictive factor for relapse.

摘要

目的

免疫球蛋白A血管炎/过敏性紫癜(IgAV/HSP)是一种累及小血管的系统性血管炎,有含IgA的免疫复合物沉积。它是儿童最常见的原发性系统性血管炎,在成人中则较少见。我们的目的是研究成人和儿童IgAV/HSP患者之间的异同。

方法

我们回顾性评估了在土耳其安卡拉哈杰泰佩大学风湿病科和儿童风湿病科就诊的35例成人和159例儿童(<18岁)临床诊断为IgAV/HSP患者的病历。儿童和成人患者分别根据2008年安卡拉标准和1990年美国风湿病学会标准诊断为IgAV/HSP。

结果

上呼吸道感染是成人(34.3%)和儿童(21.4%)常见的诱发因素。成人肌酐和C反应蛋白水平较高;诊断时皮肤活检、高血压、肾脏受累、血尿、蛋白尿和肾功能不全在成人中比儿童更常见。儿童血小板计数高于成人。儿童未经治疗随访且完全康复的情况更常见,而成人持续血尿、慢性肾衰竭、复发以及使用糖皮质激素/硫唑嘌呤的情况更常见。复发的唯一独立预测因素是持续血尿。

结论

儿童和成人IgAV/HSP患者在各种临床和实验室特征方面存在差异。总体而言,IgAV/HSP在儿童中病程呈自限性,但在成人中是一种更严重的疾病形式,肾脏受累更严重。持续血尿是复发的预测因素。

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