Claudel Sophie E, Tucker Bryan M, Kleven Daniel T, Pirkle James L, Murea Mariana
Wake Forest School of Medicine, Winston Salem, North Carolina, USA.
Department of Internal Medicine, Section of Nephrology, Baylor College of Medicine, Houston, Texas, USA.
Kidney Int Rep. 2020 Jan 13;5(5):586-599. doi: 10.1016/j.ekir.2019.12.018. eCollection 2020 May.
Pauci-immune necrotizing and crescentic glomerulonephritis (GN) is the most common etiology of rapidly progressive GN. Clinical presentation in those afflicted is usually related to rapid loss of kidney function. We report the case of a 70-year-old woman who came to medical attention for signs and symptoms related to lower-extremity deep vein thrombosis (DVT). At presentation, the patient had biochemical abnormalities consistent with active GN, which quickly progressed to rapid loss in kidney function requiring renal replacement therapy. Kidney biopsy revealed small-vessel vasculitis with glomerular crescents. Serologic studies were negative for antineutrophil cytoplasmic antibody antibodies and other causes of acute GN. Plasmapheresis, immunosuppressive, and anticoagulant therapies were prescribed. Absence of other apparent end-organ involvement with vasculitis pointed toward renal-limited small-vessel vasculitis, yet presence of DVT argues for systemic vascular inflammation. This case illustrates that venous thrombosis can be the presenting manifestation in patients with vasculitis and silent, severe end-organ involvement. The epidemiology and pathophysiology of venous thromboembolism in small-vessel vasculitis are discussed in this report.
寡免疫性坏死性新月体性肾小球肾炎（GN）是快速进展性GN最常见的病因。患者的临床表现通常与肾功能迅速丧失有关。我们报告了一例70岁女性，因下肢深静脉血栓形成（DVT）的相关症状前来就医。就诊时，患者存在与活动性GN相符的生化异常，肾功能迅速恶化，需要进行肾脏替代治疗。肾活检显示小血管血管炎伴肾小球新月体形成。血清学检查抗中性粒细胞胞浆抗体及其他急性GN病因均为阴性。给予了血浆置换、免疫抑制和抗凝治疗。无其他明显的血管炎累及终末器官表现提示为肾脏局限性小血管血管炎，但DVT的存在提示存在系统性血管炎症。该病例表明静脉血栓形成可为血管炎患者的首发表现，且存在隐匿性、严重的终末器官受累情况。本报告讨论了小血管血管炎中静脉血栓栓塞的流行病学和病理生理学。
