Talon Andrew, Tazi Nina
Internal Medicine, Creighton University School of Medicine, St. Joseph's Hospital and Medical Center, Phoenix, USA.
Internal Medicine, Dignity Health Medical Group, St. Joseph's Hospital and Medical Center, Phoenix, USA.
Cureus. 2020 Dec 18;12(12):e12155. doi: 10.7759/cureus.12155.
Renal-limited pauci-immune necrotizing glomerulonephritis presenting as acute encephalopathy is a rare occurrence. A 67-year-old Hispanic male presented to the hospital after being found down. He was found to have acute renal failure and high anion gap metabolic acidosis. Hemodialysis did not improve his encephalopathy. A vasculitis workup resulted in a high antimyeloperoxidase (MPO) antibody level. Renal biopsy revealed globally sclerotic glomeruli with focal thickened capillary loops, suggestive of pauci-immune necrotizing and crescentic glomerulonephritis (GN). Treatment consisted of high dose methylprednisolone and rituximab for induction, and three cycles of plasmapheresis, in addition to hemodialysis for uremia. Upon discharge, he was continued on hemodialysis and continued treatment with prednisone. Patients who present acutely with persistent uremic encephalopathy despite hemodialysis may warrant pursuing an alternative diagnosis, such as glomerulonephritis. Prompt diagnosis and treatment are necessary to improve the prognosis since untreated pauci-immune glomerulonephritis carries a high mortality rate.
以急性脑病为表现的肾脏局限性少免疫性坏死性肾小球肾炎是一种罕见的病症。一名67岁的西班牙裔男性在被发现晕倒后被送往医院。他被诊断为急性肾衰竭和高阴离子间隙代谢性酸中毒。血液透析并未改善他的脑病症状。血管炎检查显示抗髓过氧化物酶（MPO）抗体水平升高。肾脏活检显示肾小球全球硬化，伴有局灶性毛细血管袢增厚，提示少免疫性坏死性新月体性肾小球肾炎（GN）。治疗包括大剂量甲基强的松龙和利妥昔单抗诱导治疗，以及三个疗程的血浆置换，此外还进行血液透析治疗尿毒症。出院时，他继续接受血液透析，并继续使用泼尼松治疗。尽管进行了血液透析，但仍急性出现持续性尿毒症脑病的患者可能需要寻求其他诊断，如肾小球肾炎。由于未经治疗的少免疫性肾小球肾炎死亡率很高，因此及时诊断和治疗对于改善预后至关重要。
