Pain Clare E, Constantin Tamás, Toplak Natasa, Moll Monica, Iking-Konert Christof, Piotto Daniella P, Aktay Ayaz Nuray, Nemcova Dana, Hoeger Peter H, Cutolo Maurizio, Smith Vanessa, Foeldvari Ivan
Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
Semmelweis University, Budapest, Hungary.
Clin Exp Rheumatol. 2016 Sep-Oct;34 Suppl 100(5):200-206. Epub 2016 Jul 27.
To develop recommendations for investigation and monitoring of children with Raynaud's syndrome, based on paediatric evidence collated by a systematic review.
A systematic review was undertaken to establish the paediatric evidence for assessment and monitoring of Raynaud's syndrome. An expert panel including members of the Paediatric Rheumatology European Society (PRES) Scleroderma Working Group, were invited to a consensus meeting where recommendations were developed based on evidence graded by the systematic review and where evidence was lacking, consensus opinion. A nominal technique was used where 75% consensus was taken as agreement.
The expert panel recommended testing anti-nuclear antibody (ANA), more specific antibodies associated with connective tissue disease and nail-fold capillaroscopy in all children presenting with Raynaud's syndrome as data suggests these can be risk factors for evolution into a connective tissue disease. The frequency of follow-up recommended depends on presence of these risk factors with the aim to detect evolving connective tissue disease early in high risk individuals. Those with no abnormalities on capillaroscopy and negative autoantibodies were deemed low risk of progression, whereas those with ANA positivity, specific autoantibodies and/or nailfold capillary changes were deemed high risk and more frequent follow-up was recommended.
Recommendations, primarily based on consensus opinion, were agreed regarding investigation and monitoring of children who present with Raynaud's syndrome. Further prospective studies are needed to better define the risk factors for progression to connective tissue disease.
基于系统评价整理的儿科证据，制定对雷诺综合征患儿进行调查和监测的建议。
进行了一项系统评价，以确立评估和监测雷诺综合征的儿科证据。一个专家小组，包括欧洲儿科风湿病学会（PRES）硬皮病工作组的成员，受邀参加了一次共识会议，会上根据系统评价分级的证据以及证据不足时的共识意见制定了建议。采用了一种名义技术，将75%的共识视为达成一致。
专家小组建议，对所有出现雷诺综合征的儿童进行抗核抗体（ANA）检测、与结缔组织病相关的更特异性抗体检测以及甲襞毛细血管镜检查，因为数据表明这些可能是发展为结缔组织病的危险因素。建议的随访频率取决于这些危险因素的存在情况，目的是在高危个体中早期发现进展性结缔组织病。甲襞毛细血管镜检查无异常且自身抗体阴性的患者被认为进展风险低，而ANA阳性、特异性自身抗体阳性和/或甲襞毛细血管改变的患者被认为是高危患者，建议更频繁地随访。
就雷诺综合征患儿的调查和监测达成了主要基于共识意见的建议。需要进一步的前瞻性研究来更好地确定进展为结缔组织病的危险因素。
