Adamichou Christina, Nikolopoulos Dionysios, Papastefanakis Emmanouil, Kalogiannaki Eleni, Gergianaki Irini, Kountouri Aggeliki, Repa Argyro, Avgoustidis Nestor, Kougkas Nikolaos, Sidiropoulos Prodromos, Fanouriakis Antonis, Bertsias George
Department of Rheumatology, Clinical Immunology and Allergy, University of Crete School of Medicine, Heraklion, Greece.
Rheumatology Clinic, 'Attikon' University Hospital, Athens, Greece.
Mediterr J Rheumatol. 2018 Dec 18;29(4):232-235. doi: 10.31138/mjr.29.4.232. eCollection 2018 Dec.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by significant clinical heterogeneity with early diagnosis being a major challenge, complicated by the absence of formal diagnostic criteria. Instead, classification criteria have been developed to enable the homogenous inclusion of patients in clinical trials, with the most commonly used those of the American College of Rheumatology (ACR 1997) and the Systemic Lupus International Collaborating Clinics Classification Criteria (SLICC 2012). These criteria are widely used in clinical practice as diagnostic tools, although they fail to diagnose up to 20% of patients with SLE or may delay diagnosis. These restrictions have led to the recent (2018) introduction of new classification criteria jointly by the European League Against Rheumatism (EULAR) and ACR.
We will compare the sensitivity and specificity of the earlier and new classification criteria after a systematic analysis (retrospective study) of a group of SLE patients. In addition, we will examine which set of criteria permits the earliest classification of the disease in a prospective cohort of patients with undifferentiated connective tissue disease (UCTD). The prognostic impact (permanent organ damage) of the classification of SLE patients with the three sets of criteria will also be examined.
Data from the existing Cretan lupus registry will be used to retrospectively include consecutively registered patients aged ≥15 years diagnosed with SLE during 01/2005-12/2016 by an expert physician and followed-up for at least 6 months. All sets of criteria (ACR 1997, SLICC 2012, EULAR/ACR 2018) will be tested at the time of physician-based diagnosis and also at last follow-up. A prospective study arm will include cases with a diagnosis of UCTD and will be followed-up in the outpatient clinic for 3-5 years.
This is the first study to include the application of the new criteria (EULAR/ACR 2018) to a group of SLE patients. Determining their diagnostic value in comparison to existing criteria or diagnosis by a specialist will provide important information both for the value of their application at the level of clinical studies and for their use in clinical practice as diagnostic criteria.
系统性红斑狼疮（SLE）是一种慢性自身免疫性疾病，其临床异质性显著，早期诊断是一项重大挑战，且缺乏正式的诊断标准。取而代之的是，已制定分类标准以便在临床试验中统一纳入患者，最常用的是美国风湿病学会（1997年美国风湿病学会标准）和系统性红斑狼疮国际协作临床分类标准（2012年SLICC标准）。这些标准在临床实践中被广泛用作诊断工具，尽管它们无法诊断高达20%的SLE患者，或可能导致诊断延迟。这些局限性导致了欧洲抗风湿病联盟（EULAR）和美国风湿病学会在近期（2018年）联合推出了新的分类标准。
在对一组SLE患者进行系统分析（回顾性研究）后，我们将比较早期和新分类标准的敏感性和特异性。此外，我们将研究在哪一组标准能使未分化结缔组织病（UCTD）患者前瞻性队列中最早对疾病进行分类。还将研究使用这三组标准对SLE患者进行分类的预后影响（永久性器官损害）。
来自现有的克里特岛狼疮登记处的数据将用于回顾性纳入2005年1月至2016年12月期间由专家医生诊断为SLE且年龄≥15岁并接受至少6个月随访的连续登记患者。所有标准组（1997年美国风湿病学会标准、2012年SLICC标准、2018年EULAR/ACR标准）将在基于医生的诊断时以及最后一次随访时进行测试。前瞻性研究组将包括诊断为UCTD的病例，并将在门诊随访3至5年。
这是第一项将新的标准（2018年EULAR/ACR标准）应用于一组SLE患者的研究。与现有标准或专家诊断相比，确定其诊断价值将为其在临床研究层面的应用价值以及在临床实践中作为诊断标准的使用提供重要信息。
