University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases (ERK-net, ERN-Reconnect and RITA-ERN Member) with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID), Coordinating Center of the Interregional Network for Rare Diseases of Piedmont and Aosta Valley, San Giovanni Bosco Hub Hospital, Piazza del Donatore di Sangue 3, 10154, Turin, Italy.
Drugs. 2023 Feb;83(2):117-134. doi: 10.1007/s40265-022-01824-x. Epub 2023 Feb 2.
Systemic lupus erythematosus (SLE) is characterized by an aberrant immune response, leading to an extremely heterogeneous clinical presentation, potentially affecting different systems and organs. Despite the fact that SLE mortality has greatly decreased since the introduction of steroids, some forms of refractory/severe SLE still have the potential to result in permanent organ damage as well as increased mortality and morbidity. Furthermore, SLE patients with multiple comorbidities may face a clinical conundrum and have a bad prognosis. An improved prognosis for severe refractory SLE depends on prompt and appropriate treatment. Due to the scarcity of solid data from a well-characterized group of patients with refractory/severe SLE coming from randomized controlled studies, this review aims to shed light on this with real-world evidence from clinical research performed at our Unit, the University Center of Excellence on Nephrologic, Rheumatologic and Rare Diseases with Nephrology and Dialysis Unit and Center of Immuno-Rheumatology and Rare Diseases (CMID) (Turin, Italy). In order to determine the key clinical and prognostic features, and therapeutic approaches for severe and/or refractory SLE, our experience will be described together with existing literature, primarily focused on dermatological, neuropsychiatric, and renal symptoms.
系统性红斑狼疮(SLE)的特征是免疫反应异常,导致临床表现极其多样化,可能影响不同的系统和器官。尽管自类固醇问世以来,SLE 的死亡率已经大大降低,但某些形式的难治性/严重 SLE 仍有可能导致永久性器官损伤以及死亡率和发病率增加。此外,患有多种合并症的 SLE 患者可能面临临床难题和预后不良。改善严重难治性 SLE 的预后取决于及时和适当的治疗。由于缺乏来自难治性/严重 SLE 患者的特征明确的随机对照研究的可靠数据,本综述旨在通过我们单位、肾脏病学、风湿病学和罕见疾病卓越中心的临床研究的真实世界证据来阐明这一点,肾脏病学和透析单位以及免疫风湿病学和罕见疾病中心(CMID)(意大利都灵)。为了确定严重和/或难治性 SLE 的关键临床和预后特征以及治疗方法,我们将结合现有文献描述我们的经验,主要侧重于皮肤科、神经精神病学和肾脏症状。