Kaneko Shohei, Watanabe Eri, Abe Mai, Watanabe Shinji, Yabe Hiroki, Kojima Shigehiro, Takagi Kenji, Hirai Keiji, Morishita Yoshiyuki, Terai Chihiro
Department of Rheumatology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.
Department of Nephrology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.
J Med Case Rep. 2019 Mar 20;13(1):74. doi: 10.1186/s13256-019-1993-z.
Segmental arterial mediolysis is a rare nonarteriosclerotic and noninflammatory vascular disease that may cause intraperitoneal bleeding. Scleroderma renal crisis is a rare complication of systemic sclerosis, leading to severe hypertension and renal dysfunction. To the best of our knowledge, this is the first reported case of a patient with concurrent systemic sclerosis with scleroderma renal crisis and pathologically confirmed segmental arterial mediolysis.
We report a case of a 68-year-old Chinese woman diagnosed with systemic sclerosis who was found to have coexisting segmental arterial mediolysis. She presented with back pain, and massive intraperitoneal bleeding was detected by computed tomography. She underwent laparotomy, and the bleeding was found to originate from the gastroepiploic artery. The pathological examination demonstrated gastroepiploic arterial dissection caused by segmental arterial mediolysis. After surgery, she developed severe hypertension with hyperreninemia and progressive renal dysfunction. Given the risk factors of corticosteroid administration and the presence of anti-ribonucleic acid polymerase III antibody, she was diagnosed with scleroderma renal crisis. The patient was proved to have a very rare case of coexisting scleroderma renal crisis and segmental arterial mediolysis.
There is no known etiological connection between segmental arterial mediolysis and systemic sclerosis or scleroderma renal crisis, but it is possible that coexisting segmental arterial mediolysis and scleroderma renal crisis may have interacted to trigger the development of the other in our patient.
节段性动脉中层溶解是一种罕见的非动脉硬化性和非炎性血管疾病，可导致腹腔内出血。硬皮病肾危象是系统性硬化症的一种罕见并发症，可导致严重高血压和肾功能不全。据我们所知，这是首例报告的同时患有系统性硬化症合并硬皮病肾危象且经病理证实为节段性动脉中层溶解的患者。
我们报告一例68岁中国女性患者，诊断为系统性硬化症，同时发现存在节段性动脉中层溶解。她因背痛就诊，计算机断层扫描检测到大量腹腔内出血。她接受了剖腹手术，发现出血源自胃网膜动脉。病理检查显示胃网膜动脉夹层由节段性动脉中层溶解引起。术后，她出现严重高血压伴高肾素血症和进行性肾功能不全。鉴于使用皮质类固醇的危险因素以及抗核糖核酸聚合酶III抗体的存在，她被诊断为硬皮病肾危象。该患者被证实为同时患有硬皮病肾危象和节段性动脉中层溶解的极为罕见病例。
节段性动脉中层溶解与系统性硬化症或硬皮病肾危象之间尚无已知的病因联系，但在我们的患者中，节段性动脉中层溶解和硬皮病肾危象可能相互作用，引发了对方的发展。
