Hanna Ramy M, Abdelnour Lama, Zuckerman Jonathan E, Ferrey Antoney J, Pai Alex, Vahabzadeh Kambiz, Wilson James, Torres Everado A, Kalantar-Zadeh Kamyar, Kurtz Ira B
Division of Nephrology, Department of Medicine, University of California Irvine, Irvine, CA, USA.
UC Irvine School of Medicine, Orange, CA, USA.
SAGE Open Med Case Rep. 2020 Aug 21;8:2050313X20952650. doi: 10.1177/2050313X20952650. eCollection 2020.
Scleroderma renal crisis is a serious complication that can develop in certain patients with systemic sclerosis. Some risks have been identified as potential triggers of scleroderma renal crisis, including the high-dose oral corticosteroids. Here, we present a patient who developed clinically severe systemic sclerosis and scleroderma renal crisis after exposure to oral corticosteroids and intravitreal vascular endothelial growth factor blockade with bevacizumab for cotton wool spots. The patient's scleroderma renal crisis was severe, progressive, and refractory to the standard of care therapy: oral captopril. Biopsy showed a diffuse thrombotic microangiopathy and findings consistent with scleroderma renal crisis. We hypothesize that depletion of systemic vascular endothelial growth factor with intravitreal anti-vascular endothelial growth factor injections likely contributed to the particularly severe presentation seen in this case. Though the finding of a monoclonal gammopathy of undetermined significance is another complicating factor, this case suggests that vascular endothelial growth factor inhibition may be a newly recognized trigger of scleroderma renal crisis.
硬皮病肾危象是一种可在某些系统性硬化症患者中发生的严重并发症。一些风险已被确定为硬皮病肾危象的潜在触发因素，包括大剂量口服糖皮质激素。在此，我们报告一名患者，其在因棉絮斑接受口服糖皮质激素和玻璃体内注射贝伐单抗阻断血管内皮生长因子后，出现了临床严重的系统性硬化症和硬皮病肾危象。该患者的硬皮病肾危象严重、进行性发展且对标准治疗药物口服卡托普利无效。活检显示弥漫性血栓性微血管病，结果符合硬皮病肾危象。我们推测，玻璃体内注射抗血管内皮生长因子导致全身血管内皮生长因子耗竭可能是该病例中所见特别严重表现的原因。尽管意义未明的单克隆丙种球蛋白病这一发现是另一个复杂因素，但该病例提示血管内皮生长因子抑制可能是硬皮病肾危象一个新发现的触发因素。
