Cheng Song, Wu Sikai, Wang Neng, Xu Wenjie, Wei Fuquan, Ao Weiqun, Yuan Li, Ning Xiaoxiang, Mao Yichuan, Zhang Xianzhen, Mao Guoqun
Department of Radiology, Tongde Hospital of Zhejiang Province, Hangzhou, Zhejiang Province, China.
Zhejiang Chinese Medical University, Hangzhou, Zhejiang Province, China.
Medicine (Baltimore). 2025 Jan 3;104(1):e41208. doi: 10.1097/MD.0000000000041208.
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems. Retroperitoneal fibrosis (RPF) is a rare condition characterized by the development of fibrous tissue in the retroperitoneal space. The coexistence of SLE and RPF is extremely uncommon, and this report aims to enhance understanding of this complex relationship.
A 32-year-old woman presented with sudden-onset syncope. Her medical history revealed a 5-year history of SLE, and imaging studies identified a retroperitoneal mass.
A comprehensive diagnostic workup, including magnetic resonance imaging (MRI) and biopsy, confirmed retroperitoneal fibrosis secondary to SLE.
The patient was treated with high-dose corticosteroids, immunosuppressive therapy, and the biologic agent rituximab.
The patient's symptoms markedly improved, and follow-up MRI demonstrated significant regression of the retroperitoneal lesion.
RPF associated with SLE is exceptionally rare. This case underscores the importance of early diagnosis and a coordinated multidisciplinary approach in managing such complex conditions. Glucocorticoid therapy remains the cornerstone of treatment, augmented by immunosuppressants and biologic agents when necessary.
系统性红斑狼疮(SLE)是一种影响多个器官系统的慢性自身免疫性疾病。腹膜后纤维化(RPF)是一种罕见病症,其特征为腹膜后间隙出现纤维组织。SLE与RPF并存极为罕见,本报告旨在增进对这种复杂关系的理解。
一名32岁女性因突发晕厥就诊。她有5年SLE病史,影像学检查发现腹膜后肿块。
包括磁共振成像(MRI)和活检在内的全面诊断检查确诊为继发于SLE的腹膜后纤维化。
患者接受了大剂量糖皮质激素、免疫抑制治疗以及生物制剂利妥昔单抗治疗。
患者症状明显改善,随访MRI显示腹膜后病变显著消退。
与SLE相关的RPF极为罕见。该病例强调了早期诊断以及在处理此类复杂病症时采用多学科协作方法的重要性。糖皮质激素治疗仍然是治疗的基石,必要时可辅以免疫抑制剂和生物制剂。