EULAR/PRINTO/PRES 儿童过敏性紫癜、结节性多动脉炎、韦格纳肉芽肿和川崎病的分类标准：安卡拉 2008 年标准。第一部分：整体方法和临床特征。

EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation.

机构信息

Paediatric Rheumatology International Trials Organisation (PRINTO), IRCCS G Gaslini, Università di Genova, Pediatria II - Reumatologia, EULAR Centre of Excellence in Rheumatology 2008-2013, Largo Gaslini, 5, Genova 16147, Italy.

出版信息

Ann Rheum Dis. 2010 May;69(5):790-7. doi: 10.1136/ard.2009.116624. Epub 2010 Apr 13.

DOI:10.1136/ard.2009.116624

PMID:20388738

Abstract

OBJECTIVES

To report methodology and overall clinical, laboratory and radiographic characteristics for Henoch-Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) classification criteria.

METHODS

The preliminary Vienna 2005 consensus conference, which proposed preliminary criteria for paediatric vasculitides, was followed by a EULAR/PRINTO/PRES - supported validation project divided into three main steps. Step 1: retrospective/prospective web-data collection for HSP, c-PAN, c-WG and c-TA, with age at diagnosis <or=18 years. Step 2: blinded classification by consensus panel of a subgroup of 280 cases (128 difficult cases, 152 randomly selected) enabling expert diagnostic verification. Step 3: Ankara 2008 Consensus Conference and statistical evaluation (sensitivity, specificity, area under the curve, kappa-agreement) using as 'gold standard' the final consensus classification or original treating physician diagnosis.

RESULTS

A total of 1183/1398 (85%) samples collected were available for analysis: 827 HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms, laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides. A representative subgroup of 280 patients was blinded to the treating physician diagnosis and classified by a consensus panel, with a kappa-agreement of 0.96 for HSP (95% CI 0.84 to 1), 0.88 for c-WG (95% CI 0.76 to 0.99), 0.84 for c-TA (95% CI 0.73 to 0.96) and 0.73 for c-PAN (95% CI 0.62 to 0.84), with an overall kappa of 0.79 (95% CI 0.73 to 0.84).

CONCLUSION

EULAR/PRINTO/PRES propose validated classification criteria for HSP, c-PAN, c-WG and c-TA, with substantial/almost perfect agreement with the final consensus classification or original treating physician diagnosis.

摘要

目的

报告亨诺克-舒恩莱因紫癜（HSP）、儿童多发性动脉炎（c-PAN）、c-Wegener 肉芽肿（c-WG）和 c-Takayasu 动脉炎（c-TA）分类标准的方法学和总体临床、实验室及影像学特征。

方法

初步的维也纳 2005 年共识会议提出了儿科血管炎的初步标准，随后开展了一项由 EULAR/PRINTO/PRES 支持的验证项目，分为三个主要步骤。步骤 1：通过回顾性/前瞻性网络数据收集 HSP、c-PAN、c-WG 和 c-TA，诊断时年龄≤18 岁。步骤 2：由共识小组对 280 例（128 例疑难病例，152 例随机选择）进行盲法分类，实现专家诊断验证。步骤 3：在安卡拉 2008 年共识会议上，使用最终共识分类或原始治疗医生诊断作为“金标准”进行统计评估（敏感性、特异性、曲线下面积、kappa 一致性）。

结果

共分析了 1183/1398（85%）例可获得的样本：827 例 HSP、150 例 c-PAN、60 例 c-WG、87 例 c-TA 和 59 例 c-其他。发病率、体征/症状、实验室、活检和影像学报告与四种 c-血管炎的临床表现一致。对 280 例代表性患者进行盲法治疗医生诊断，并由共识小组进行分类，HSP 的 kappa 一致性为 0.96（95% CI 0.84 至 1），c-WG 为 0.88（95% CI 0.76 至 0.99），c-TA 为 0.84（95% CI 0.73 至 0.96），c-PAN 为 0.73（95% CI 0.62 至 0.84），总体 kappa 值为 0.79（95% CI 0.73 至 0.84）。