目的:自2012年意大利风湿病学会(SIR)认可的原发性大血管血管炎(LVV)的最新建议发表以来,出现了有关常规和生物免疫抑制药物的诊断和治疗的新证据。临床护理的相关潜在变化支持了更新原始建议的需要。
方法:使用建议评估的分级,开发和评估(等级)-ADOLOPMENT框架,我们进行了系统的文献综述,以更新支持欧洲风湿病学协会联盟(EULAR)LVV指南的证据.由12名临床专家组成的多学科小组,训练有素的护士,一名患者代表与证据审查小组合作讨论了该建议。咨询了61个利益攸关方,以外部审查和评价建议。
结果:制定了12项建议。怀疑诊断为LVV应通过影像学或组织学证实。在主动GCA或TAK中,强烈建议立即开始口服大剂量糖皮质激素(相当于每天40~60mg泼尼松),以诱导临床缓解.在选定的GCA患者中(例如,难治性或复发性疾病或有糖皮质激素相关不良反应风险的患者)建议使用辅助治疗(托珠单抗或甲氨蝶呤)。在所有诊断为TAK的患者中,辅助疗法,如常规合成或生物免疫抑制剂,应与糖皮质激素联合使用。
结论:制定了一套新的SIR建议,以便为怀疑或明确诊断为LVV的患者的诊断和治疗提供指导。
OBJECTIVE: Since of the last publication of last recommendations on primary large-vessel vasculitis (LVV) endorsed by the Italian Society of Rheumatology (SIR) in 2012, new evidence emerged regarding the diagnosis and the treatment with conventional and biologic immunosuppressive drugs. The associated potential change of clinical care supported the need to update the original recommendations.
METHODS: Using the grading of recommendations assessment, development and evaluation (GRADE)-ADOLOPMENT framework, a systematic literature review was performed to update the evidence supporting the European Alliance of Associations for Rheumatology (EULAR)
guidelines on LVV as reference. A multidisciplinary panel of 12 expert clinicians, a trained nurse, and a patients\' representative discussed the recommendation in cooperation with an Evidence Review Team. Sixty-one stakeholders were consulted to externally review and rate the recommendations.
RESULTS: Twelve recommendations were formulated. A suspected diagnosis of LVV should be confirmed by imaging or histology. In active GCA or TAK, the prompt commencement of high dose of oral glucocorticoids (40-60 mg prednisone-equivalent per day) is strongly recommended to induce clinical remission. In selected patients with GCA (e.g., refractory or relapsing disease or patients at risk of glucocorticoid related adverse effects) the use of an adjunctive therapy (tocilizumab or methotrexate) is recommended. In all patients diagnosed with TAK, adjunctive therapies, such as conventional synthetic or biological immunosuppressants, should be given in combination with glucocorticoids.
CONCLUSIONS: The new set of SIR recommendations was formulated in order to provide a guidance on both diagnosis and treatment of patients suspected of or with a definite diagnosis of LVV.