Iaccarino Luca, Talarico Rosaria, Scirè Carlo Alberto, Amoura Zahir, Burmester Gerd, Doria Andrea, Faiz Karim, Frank Charissa, Hachulla Eric, Hie Miguel, Launay David, Montecucco Carlomaurizio, Monti Sara, Mouthon Luc, Tincani Angela, Toniati Paola, Van Hagen Pieter Martin, Van Vollenhoven Ronald F, Bombardieri Stefano, Mueller-Ladner Ulf, Schneider Matthias, Smith Vanessa, Cutolo Maurizio, Mosca Marta, Alexander Tobias
Rheumatology Unit, Department of Medicine, AO Padova and University of Padua, Padua, Italy.
Rheumatology Unit, AOU Pisana, Pisa, Italy.
RMD Open. 2019 Jan 19;4(Suppl 1):e000787. doi: 10.1136/rmdopen-2018-000787. eCollection 2018.
Immunoglobulin G4-related diseases (IgG4-RD) are a group of chronic relapsing-remitting inflammatory conditions, characterised by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, fibrosis and a usually favourable response to steroids. In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the European Reference Network ReCONNET, aimed at evaluating existing clinical practice guidelines (CPGs) and recommendations in IgG4-RD. From 167 publications initially obtained from a systematic literature search, only one was identified as a systematic multispecialist, evidence-based, consensus guidance statement on diagnosis and treatment of IgG4-RD, which may be recommended for use as CPG in IgG4-RD. With the recognition of a limited evidence based in this increasingly recognised disease, the group discussion has identified the following unmet needs: lack of shared classification criteria, absence of formal guidelines on diagnosis, no evidence-based therapeutic recommendations and lack of activity and damage indices. Areas of unmet needs include the difficulties in diagnosis, management and monitoring and the scarcity of expert centres.
免疫球蛋白G4相关疾病（IgG4-RD）是一组慢性复发-缓解性炎症性疾病，其特征为淋巴细胞和分泌IgG4的浆细胞浸润组织、纤维化，且通常对类固醇治疗反应良好。在本叙述性综述中，我们总结了一项系统文献研究的结果，该研究作为欧洲参考网络ReCONNET的一部分进行，旨在评估IgG4-RD的现有临床实践指南（CPG）和建议。从系统文献检索最初获得的167篇出版物中，仅一篇被确定为关于IgG4-RD诊断和治疗的系统多专科、基于证据的共识指导声明，可推荐用作IgG4-RD的CPG。鉴于在这种日益被认识的疾病中基于证据有限，小组讨论确定了以下未满足的需求：缺乏共享的分类标准、缺乏正式的诊断指南、没有基于证据的治疗建议以及缺乏活动和损伤指标。未满足需求的领域包括诊断、管理和监测方面的困难以及专家中心的稀缺。
