Characterization of systemic disease in primary Sjogren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements
Characterization of systemic disease in primary Sjogren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements
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DOI：
10.1093/rheumatology/kev200
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发表时间：
2015-12-01
期刊：
影响因子：
5.5
通讯作者：
Vitali, Claudio
中科院分区：
文献类型：
--
作者：
Ramos-Casals, Manuel;Brito-Zeron, Pilar;Vitali, Claudio
客观的。就原发性 SS 中主要器官特异性腺外表现的定义和特征达成欧洲共识。方法。 EULAR-SS 工作组指导委员会同意根据 EULAR SS 疾病活动指数 (ESSDAI) 分类来处理 SS 相关的全身受累，并建议准备四份单独的手稿：关节、皮肤、肺和肾脏 ESSDAI 受累；肌肉、周围神经系统、中枢神经系统和血液 ESSDAI 受累；未纳入 ESSDAI 分类的器官；和淋巴组织增生性疾病。目前可用的证据是通过系统文献综述获得的，重点关注 SS 相关的系统特征。结果。总结了关节、皮肤、肺和肾脏受累的以下信息：临床特征的明确、一致的定义、简要的流行病学描述，包括对主要临床系列中报告的患病率的估计，以及可以帮助医生清楚识别这些特征的关键临床和诊断特征的简要列表。不幸的是，我们发现证据主要依赖于从个别案例中检索到的信息，并且提供的科学信息是异构的。由于重症病例与非重症病例的报告不平衡，对受累类型的分析存在偏差，尽管偏差的主要来源是器官受累的异质定义（甚至某些研究中缺乏定义）以及调查每个器官受累的研究中使用的异质诊断方法。结论。本文中的建议是开发原发性 SS 全身受累最佳诊断方法的第一步，并可能为进一步制定循证诊断和治疗指南铺平道路。
Objective. To reach a European consensus on the definition and characterization of the main organspecific extraglandular manifestations in primary SS.Methods. The EULAR-SS Task Force Group steering committee agreed to approach SS-related systemic involvement according to the EULAR SS Disease Activity Index (ESSDAI) classification and proposed the preparation of four separate manuscripts: articular, cutaneous, pulmonary and renal ESSDAI involvement; muscular, peripheral nervous system, CNS and haematological ESSDAI involvement; organs not included in the ESSDAI classification; and lymphoproliferative disease. Currently available evidence was obtained by a systematic literature review focused on SS-related systemic features.Results. The following information was summarized for articular, cutaneous, pulmonary and renal involvement: a clear, consensual definition of the clinical feature, a brief epidemiological description including an estimate of the prevalence reported in the main clinical series and a brief list of the key clinical and diagnostic features that could help physicians clearly identify these features. Unfortunately we found that the body of evidence relied predominantly on information retrieved from individual cases, and the scientific information provided was heterogeneous. The analysis of types of involvement was biased due to the unbalanced reporting of severe cases over non-severe cases, although the main sources of bias were the heterogeneous definitions of organ involvement (or even the lack of definition in some studies) and the heterogeneous diagnostic approach used in studies to investigate involvment of each organ.Conclusion. The proposals included in this article are a first step to developing an optimal diagnostic approach to systemic involvement in primary SS and may pave the way for further development of evidence-based diagnostic and therapeutic guidelines.