Department of Medical Sciences and Public Health, University of Cagliari, Italy; Rheumatology Unit, University Clinic, AOU, Cagliari, Italy.
McMaster Lupus Clinic, Department of Medicine, McMaster University, Toronto, Canada.
Best Pract Res Clin Rheumatol. 2023 Dec;37(4):101938. doi: 10.1016/j.berh.2024.101938. Epub 2024 Feb 22.
Systemic lupus erythematosus (SLE) is a complex disease with an insidious clinical presentation. In up to half of the cases, SLE onset is characterized by clinical and serological manifestations that, although specific, are insufficient to fulfill the classification criteria. This condition, called incomplete SLE, could be as challenging as the definite and classifiable SLE and requires to be treated according to the severity of clinical manifestations. In addition, an early SLE diagnosis and therapeutic intervention can positively influence the disease outcome, including remission rate and damage accrual. After diagnosis, the disease course is relapsing-remitting for most patients. Time in remission and cumulative glucocorticoid exposure are the most important factors for prognosis. Therefore, timely identification of SLE clinical patterns may help tailor the therapeutic intervention to the disease course. Late-onset SLE is rare but more often associated with delayed diagnosis and a higher incidence of comorbidities, including Sjogren's syndrome. This review focuses on the SLE disease course, providing actionable strategies for early diagnosis, an overview of the possible clinical patterns of SLE, and the clinical variation associated with the different age-at-onset SLE groups.
系统性红斑狼疮(SLE)是一种具有隐匿性临床表现的复杂疾病。在多达一半的病例中,SLE 的发病特征为具有特异性但不足以满足分类标准的临床和血清学表现。这种称为不典型性 SLE 的情况可能与明确且可分类的 SLE 一样具有挑战性,需要根据临床表现的严重程度进行治疗。此外,早期 SLE 的诊断和治疗干预可以积极影响疾病结局,包括缓解率和累积损伤。大多数患者的疾病过程为复发缓解型。缓解时间和累积糖皮质激素暴露是预后的最重要因素。因此,及时识别 SLE 的临床模式可能有助于根据疾病过程调整治疗干预。晚发性 SLE 较为罕见,但更常与延迟诊断和更高的合并症发生率相关,包括干燥综合征。这篇综述重点关注 SLE 的疾病过程,提供了早期诊断的可行策略、SLE 可能的临床模式概述以及与不同发病年龄组 SLE 相关的临床差异。