Center of Research of Immunopathology and Rare Diseases-Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, and SCDU Nephrology and Dialysis (ERNnet Member), San Giovanni Bosco Hospital, Turin, Italy.
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
Nat Rev Rheumatol. 2022 Jan;18(1):9-21. doi: 10.1038/s41584-021-00710-2. Epub 2021 Nov 11.
Systemic lupus erythematosus (SLE) is an autoimmune disease with heterogeneous clinical manifestations that can potentially affect every organ and system. SLE is usually identified on the basis of clinical or serological manifestations; however, some individuals can present with signs and symptoms that are consistent with SLE but are not sufficient for a definite diagnosis. Disease in these individuals can either progress over time to definite SLE or remain stable, in which case their disease is often described as intermediate, possible or probable SLE. Alternatively, such individuals might have undifferentiated connective tissue disease (UCTD). Being able to differentiate between those with stable UCTD and those with SLE at an early stage is important to avoid irreversible target-organ damage from occurring. This Review provides insight into existing and evolving perceptions of the early stages of SLE, including clinical and mechanistic considerations, as well as potential paths towards early identification and intervention. Further research into the earliest phases of SLE will be important for the development of targeted diagnostic approaches and biomarkers for the identification of individuals with early disease who are likely to progress to definite SLE.
系统性红斑狼疮（SLE）是一种具有异质性临床表现的自身免疫性疾病，可能会对每个器官和系统造成影响。SLE 的诊断通常基于临床或血清学表现；然而，一些患者可能会出现符合 SLE 的症状和体征，但不足以做出明确诊断。这些患者的疾病可能会随着时间的推移进展为明确的 SLE，也可能保持稳定，在这种情况下，其疾病通常被描述为中间型、可能型或很可能型 SLE。或者，这些患者可能患有未分化结缔组织病（UCTD）。区分稳定的 UCTD 患者和早期 SLE 患者非常重要，这样可以避免发生不可逆的靶器官损伤。这篇综述深入探讨了 SLE 早期阶段的现有和不断发展的认识，包括临床和机制方面的考虑，以及早期识别和干预的潜在途径。进一步研究 SLE 的最早阶段对于开发针对早期疾病的靶向诊断方法和生物标志物非常重要，这些方法和生物标志物可以识别可能进展为明确 SLE 的患者。
