Shen Anna, Shen Li, Li Wenyan, Cai Huaiqing, Zheng Weihua, Huang Wenyan, Zhang Rufang, Kang Yulin
Department of Nephrology and Rheumatology, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Department of Cardiothoracic Surgery, Shanghai Children's Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Front Med (Lausanne). 2025 Sep 8;12:1567332. doi: 10.3389/fmed.2025.1567332. eCollection 2025.
IgA nephropathy (IgAN) is one of the most common glomerulonephritis characterized by deposition of IgA immune complex in the mesangial region and mesangial proliferation in children. It could progress to end stage renal disease. The underlying mechanism of IgAN is not fully understood. It has been known that IgAN could be secondary to autoimmune disorders, respiratory tract diseases, neoplasia, infection, gastrointestinal and liver diseases. Midaortic syndrome (MAS) is characterized by narrowing or occlusion of the distal thoracoabdominal aorta and the openings of its major branches, which may lead to organ damage like heart failure, renal dysfunction and even death. It may be congenital (present at birth) or acquired later in life. So far, no cases of IgAN concomitant with MAS have been reported.
A 12-year-old boy was diagnosed with biopsy-proven IgAN, which was characterized by heavy proteinuria and hematuria. Additionally, MAS was found by performing abdominal computed tomography angiography (CTA) due to blood pressure difference between upper arm and thigh. Methylprednisolone and cyclophosphamide were used for treating IgAN. MAS was corrected with GORE-TEX Vascular Grafts (expanded polytetrafluoroethylene, ePTFE). Remission was achieved eventually. To the best of our knowledge, we are the first to report a childhood case who has IgAN concomitant with congenital MAS. We also presented a case-based review regarding the association between IgAN and vascular stenosis.
MAS is an aggravating factor and might be a new cause of secondary IgAN.
IgA肾病（IgAN）是最常见的肾小球肾炎之一，其特征为儿童系膜区IgA免疫复合物沉积和系膜增生。它可进展为终末期肾病。IgAN的潜在机制尚未完全明确。已知IgAN可能继发于自身免疫性疾病、呼吸道疾病、肿瘤、感染、胃肠道和肝脏疾病。中段主动脉综合征（MAS）的特征是胸腹主动脉远端及其主要分支开口狭窄或闭塞，这可能导致器官损害，如心力衰竭、肾功能不全甚至死亡。它可能是先天性的（出生时即存在）或后天获得的。迄今为止，尚无IgAN合并MAS的病例报道。
一名12岁男孩经活检证实为IgAN，其特征为大量蛋白尿和血尿。此外，由于上臂和大腿血压差异，通过腹部计算机断层扫描血管造影（CTA）发现了MAS。甲基强的松龙和环磷酰胺用于治疗IgAN。使用GORE-TEX血管移植物（膨体聚四氟乙烯，ePTFE）纠正MAS。最终实现缓解。据我们所知，我们是首个报道儿童IgAN合并先天性MAS病例的。我们还基于病例对IgAN与血管狭窄之间的关联进行了综述。
MAS是一个加重因素，可能是继发性IgAN的新病因。
