Gerosa Maria, Beretta Lorenzo, Ramirez Giuseppe Alvise, Bozzolo Enrica, Cornalba Martina, Bellocchi Chiara, Argolini Lorenza Maria, Moroni Luca, Farina Nicola, Segatto Giulia, Dagna Lorenzo, Caporali Roberto
Research Centre for Adult and Pediatric Rheumatic Diseases, Department of Clinical Sciences and Community Health, University of Milan, 20129 Milan, Italy.
ASST Pini CTO, Lupus Clinic, Division of Clinical Rheumatology, 20122 Milan, Italy.
J Clin Med. 2022 Jun 22;11(13):3587. doi: 10.3390/jcm11133587.
Tackling active disease to prevent damage accrual constitutes a major goal in the management of patients with systemic lupus erythematosus (SLE). Patients with early onset disease or in the early phase of the disease course are at increased risk of developing severe manifestations and subsequent damage accrual, while less is known about the course of the disease in the long term. To address this issue, we performed a multicentre retrospective observational study focused on patients living with SLE for at least 20 years and determined their disease status at 15 and 20 years after onset and at their last clinical evaluation. Disease activity was measured through the British Isles Lupus Assessment Group (BILAG) tool and late flares were defined as worsening in one or more BILAG domains after 20 years of disease. Remission was classified according to attainment of lupus low-disease-activity state (LLDAS) criteria or the Definitions Of Remission In SLE (DORIS) parameters. Damage was quantitated through the Systemic Lupus Erythematosus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR-DI). LLAS/DORIS remission prevalence steadily increased over time. In total, 84 patients had a late flare and 88 had late damage accrual. Lack of LLDAS/DORIS remission status at the 20 year timepoint ( = 0.0026 and = 0.0337, respectively), prednisone dose ≥ 7.5 mg ( = 9.17 × 10) or active serology (either dsDNA binding, low complement or both; = 0.001) were all associated with increased late flare risk. Late flares, in turn, heralded the development of late damage ( = 2.7 × 10). These data suggest that patients with longstanding SLE are frequently in remission but still at risk of disease flares and eventual damage accrual, suggesting the need for tailored monitoring and therapeutic approaches aiming at effective immunomodulation besides immunosuppression, at least by means of steroids.
控制活动性疾病以防止损害累积是系统性红斑狼疮（SLE）患者管理的主要目标。早发性疾病患者或疾病病程早期的患者出现严重表现和随后损害累积的风险增加，而关于该疾病的长期病程了解较少。为了解决这个问题，我们进行了一项多中心回顾性观察研究，重点关注患有SLE至少20年的患者，并确定他们在发病后15年和20年以及最后一次临床评估时的疾病状态。通过不列颠群岛狼疮评估组（BILAG）工具测量疾病活动度，晚期发作定义为疾病20年后一个或多个BILAG领域病情恶化。根据狼疮低疾病活动状态（LLDAS）标准或SLE缓解定义（DORIS）参数对缓解进行分类。通过系统性红斑狼疮国际协作临床/美国风湿病学会损害指数（SLICC/ACR-DI）对损害进行量化。LLAS/DORIS缓解患病率随时间稳步增加。共有84例患者出现晚期发作，88例出现晚期损害累积。在20年时间点缺乏LLDAS/DORIS缓解状态（分别为 = 0.0026和 = 0.0337）、泼尼松剂量≥7.5 mg（ = 9.17×10）或血清学活动（双链DNA结合、低补体或两者兼有； = 0.001）均与晚期发作风险增加相关。反过来，晚期发作预示着晚期损害的发生（ = 2.7×10）。这些数据表明，长期患有SLE的患者经常处于缓解状态，但仍有疾病发作和最终损害累积的风险，这表明除了免疫抑制（至少通过类固醇）外，还需要针对有效的免疫调节采取量身定制的监测和治疗方法。
