Rednic Simona, Damian Laura, Talarico Rosaria, Scirè Carlo Alberto, Tobias Alexander, Costedoat-Chalumeau Nathalie, Launay David, Mathian Alexis, Mattews Lisa, Ponte Cristina, Toniati Paola, Bombardieri Stefano, Frank Charissa, Schneider Matthias, Smith Vanessa, Cutolo Maurizio, Mosca Marta, Arnaud Laurent
Department of Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania.
Rheumatology Unit, AOU Pisana, Pisa, Italy.
RMD Open. 2018 Oct 18;4(Suppl 1):e000788. doi: 10.1136/rmdopen-2018-000788. eCollection 2018.
Due to the rarity of relapsing polychondritis (RP), many unmet needs remain in the management of RP. Here, we present a systematic review of clinical practice guidelines (CPGs) published for RP, as well as a list of the most striking unmet needs for this rare disease. We carried out a systematic search in PubMed and Embase based on controlled terms (medical subject headings and Emtree) and keywords of the disease and publication type (CPGs). The systematic literature review identified 20 citations, among which no CPGs could be identified. We identified 11 main areas with unmet needs in the field of RP: the diagnosis strategy for RP; the therapeutic management of RP; the management of pregnancy in RP; the management of the disease in specific age groups (for instance in paediatric-onset RP); the evaluation of adherence to treatment; the follow-up of patients with RP, including the frequency of screening for the potential complications and the optimal imaging tools for each involved region; perioperative and anaesthetic management (due to tracheal involvement); risk of neoplasms in RP, including haematological malignancies; the prevention and management of infections; tools for assessment of disease activity and damage; and patient-reported outcomes and quality of life indicators. Patients and physicians should work together within the frame of the ReCONNET network to derive valuable evidence for obtaining literature-informed CPGs.
由于复发性多软骨炎（RP）较为罕见，RP的管理仍存在许多未满足的需求。在此，我们对已发表的RP临床实践指南（CPG）进行了系统综述，并列出了这种罕见疾病最突出的未满足需求清单。我们基于受控词（医学主题词和Emtree）以及疾病关键词和出版物类型（CPG）在PubMed和Embase中进行了系统检索。系统文献综述共识别出20篇文献，但未找到CPG。我们确定了RP领域11个存在未满足需求的主要方面：RP的诊断策略；RP的治疗管理；RP患者的妊娠管理；特定年龄组（如儿童期发病的RP）的疾病管理；治疗依从性评估；RP患者的随访，包括潜在并发症筛查频率以及每个受累区域的最佳影像学检查工具；围手术期和麻醉管理（由于气管受累）；RP患者发生肿瘤的风险，包括血液系统恶性肿瘤；感染的预防和管理；疾病活动度和损伤评估工具；以及患者报告的结局和生活质量指标。患者和医生应在ReCONNET网络框架内共同努力，以获取有价值的证据，从而制定基于文献的CPG。
