Department of Internal Medicine, Institute E3M, CIMI-Paris, Faculty of medicine, National Reference Centre of Systemic Lupus, Antiphospholipid Syndrome, and Other Autoimmune Diseases, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, boulevard de l'Hôpital, 75013 Paris, France.
Paediatric Immunology and Rhumatologie, Hospital Necker for Sick Children, AP-HP, Paris, France.
Rev Med Interne. 2024 Sep;45(9):559-599. doi: 10.1016/j.revmed.2024.07.006. Epub 2024 Aug 26.
Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice. Patients are informed about routine checkups, treatment side effects, and the need for regular vaccinations, especially if they are receiving immunosuppressive treatment. They are also advised on lifestyle factors such as the risks of smoking, sun exposure, and dietary adjustments, especially when they are receiving corticosteroids. The importance of contraception, particularly when teratogenic medications are being used, and regular cancer screening are emphasized. Support networks can help relieve a patient's isolation. The first-line medical treatment of SLE is hydroxychloroquine (HCQ), possibly combined with an immunosuppressant and/or low-dose corticosteroid therapy. The treatment of flares depends on their severity, and typically involves HCQ and NSAIDs, but may be escalated to corticosteroid therapy with immunosuppressants or biologic therapies in moderate to severe cases. Because there is no curative treatment, the goals of therapy are patient comfort, preventing progression and flares, and preserving overall long-term health and fertility. The frequency of follow-up visits depends on disease severity and any new symptoms. Regular specialized assessments are necessary, especially when treatment changes, but a frequency of every 3 to 6 months is recommended during periods of remission and monthly during active or severe disease, especially in children. These assessments include both clinical and laboratory tests to monitor complications and disease activity, with specific attention to proteinuria.
由于红斑狼疮（SLE）是一种罕见疾病，并且早期管理对预后有重大影响，因此建议由具有 SLE 经验的医生进行诊断，例如来自专家中心的医生。一旦确诊，特别应确定现有的表现，评估蛋白尿以确定肾脏受累，确定疾病活动度和严重程度，预测潜在并发症，寻找相关疾病，并注意患者的社会职业和家庭背景。SLE 的治疗管理包括患者对识别症状的教育，了解疾病进展以及何时应寻求医疗建议。告知患者有关常规检查、治疗副作用以及定期接种疫苗的信息，尤其是在接受免疫抑制治疗的情况下。还建议患者注意生活方式因素，如吸烟、暴露于阳光和饮食调整的风险，尤其是在接受皮质类固醇治疗时。强调避孕的重要性，尤其是在使用致畸药物时，以及定期进行癌症筛查。支持网络可以帮助减轻患者的孤立感。SLE 的一线治疗药物是羟氯喹（HCQ），可能与免疫抑制剂和/或低剂量皮质类固醇联合使用。发作的治疗取决于其严重程度，通常包括 HCQ 和 NSAIDs，但在中度至重度情况下可能升级为皮质类固醇治疗联合免疫抑制剂或生物疗法。由于没有治愈方法，因此治疗的目标是患者的舒适度、预防疾病进展和发作以及保持整体长期健康和生育能力。随访就诊的频率取决于疾病的严重程度和任何新症状。需要定期进行专门评估，尤其是在治疗改变时，但建议在缓解期每 3 至 6 个月进行一次评估，在活动期或严重疾病期间每月进行一次评估，尤其是在儿童中。这些评估包括监测并发症和疾病活动度的临床和实验室检查，特别注意蛋白尿。
