Swaak A J, van de Brink H, Smeenk R J, Manger K, Kalden J R, Tosi S, Marchesoni A, Domljan Z, Rozman B, Logar D, Pokorny G, Kovacs L, Kovacs A, Vlachoyiannopoulos P G, Moutsopoulos H M, Chwalinska-Sadowska H, Dratwianka B, Kiss E, Cikes N, Anic B, Schneider M, Fischer R, Bombardieri S, Mosca M, Graninger W, Smolen J S
Department of Rheumatology, Zuiderziekenhuis, Groene Hilledijk 315, 3075 EA Rotterdam, The Netherlands.
Rheumatology (Oxford). 2001 Jan;40(1):89-94. doi: 10.1093/rheumatology/40.1.89.
Patients characterized with antinuclear antibodies (ANA) and disease symptoms related to one organ system can be described as having incomplete systemic lupus erythematosus (SLE). The aim of this multicentre study was to describe the outcome of these so-called incomplete SLE patients. Two aspects of the outcome were studied: (i) the disease course, defined by the presence or absence of clinical symptoms; and (ii) the number of patients that eventually developed full SLE.
Outcome parameters were the ACR criteria, the SLE disease Activity Index (SLEDAI), the European Consensus Lupus Activity Measure (ECLAM) and the requirement for treatment. In 10 European rheumatology centres, patients who had been evaluated in the last 3 months of 1994 and had been diagnosed as having incomplete SLE on clinical grounds for at least 1 yr were included in the study. All 122 patients who were included in the study were evaluated annually during 3 yr of follow-up.
Our results are confined to a patient cohort defined by disease duration of at least 1 yr, being under clinical care at the different centres in Europe. These patients showed disease activity that was related mostly to symptoms of the skin and the musculoskeletal system, and leucocytopenia. During the follow-up, low doses of prednisolone were still being prescribed in 43% of the patients. On recruitment to the study, 22 of the 122 incomplete SLE patients already fulfilled the ACR criteria for the diagnosis of SLE. In the 3 yr of follow-up only three patients developed SLE.
A high proportion of patients in our cohort defined on clinical grounds as having incomplete SLE eventually showed disease activity defined by the SLEDAI as well as ECLAM. However, only three cases developed to SLE during the follow-up. This suggests that incomplete SLE forms a subgroup of SLE that has a good prognosis.
以抗核抗体（ANA）及与一个器官系统相关的疾病症状为特征的患者可被描述为患有不完全系统性红斑狼疮（SLE）。这项多中心研究的目的是描述这些所谓不完全SLE患者的转归情况。研究了转归的两个方面：（i）疾病进程，由临床症状的有无界定；（ii）最终发展为完全SLE的患者数量。
转归参数包括美国风湿病学会（ACR）标准、SLE疾病活动指数（SLEDAI）、欧洲狼疮活动度共识评估（ECLAM）及治疗需求。在10个欧洲风湿病中心，纳入了在1994年最后3个月接受评估且基于临床诊断为不完全SLE至少1年的患者。纳入研究的所有122例患者在3年随访期间每年接受评估。
我们的结果限于一个疾病病程至少1年、在欧洲不同中心接受临床护理的患者队列。这些患者的疾病活动主要与皮肤和肌肉骨骼系统症状以及白细胞减少有关。在随访期间，43%的患者仍在使用低剂量泼尼松龙。在纳入研究时，122例不完全SLE患者中有22例已符合ACR的SLE诊断标准。在3年随访期间，只有3例患者发展为SLE。
我们队列中基于临床诊断为不完全SLE的患者中，很大一部分最终表现出由SLEDAI及ECLAM界定的疾病活动。然而，随访期间只有3例发展为SLE。这表明不完全SLE构成了SLE的一个预后良好的亚组。
