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A Challenging Twist in Pulmonary Renal Syndrome

机译:肺肾综合征的挑战性转折

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摘要

Case. We report a rare case of hydralazine-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. A 75-year-old African American woman with history of high blood pressure on hydralazine for 3 years presented with acute onset of shortness of breath and hemoptysis. Lab workup revealed a severe normocytic anemia and a serum creatinine of 5.09 mg/dL (baseline 0.9). Bronchoscopy demonstrated active pulmonary hemorrhage. A urine sample revealed red cell casts and a renal biopsy demonstrated pauci-immune, focally necrotizing glomerulonephritis with small crescents consistent with possible anti-neutrophil cytoplasmic antibody-positive renal vasculitis. Serologies showed high-titer MPO-ANCA and high-titer anti-histone antibodies. She was treated with intravenous steroids and subsequently with immunosuppression after cessation of hydralazine. The patient was subsequently discharged from hospital after a rapid clinical improvement. Conclusion. Hydralazine-induced anti-neutrophil cytoplasmic antibody-positive renal vasculitis is a rare adverse effect and can present as a severe vasculitic syndrome with multiple organ involvement. Features of this association include the presence of high titer of anti-myeloperoxidase anti-neutrophil cytoplasmic antibody with multiantigenicity, positive anti-histone antibodies, and the lack of immunoglobulin and complement deposition. Prompt cessation of hydralazine may be sufficient to reverse disease activity but immunosuppression may be needed.
机译:案件。我们报告了肼苯哒嗪诱导的抗中性粒细胞胞浆抗体相关性血管炎的罕见病例。一位75岁的非裔美国女性因服用肼苯哒嗪高血压已有3年,出现呼吸急促和咯血。实验室检查发现严重的正常红细胞性贫血和血清肌酐为5.09μmg/ dL(基线0.9)。支气管镜检查显示活动性肺出血。尿液样本显示有红细胞铸型,肾活检证实为弱免疫性,局部坏死性肾小球肾炎,小月牙,可能与抗中性粒细胞胞浆抗体阳性的肾血管炎一致。血清学检查显示高滴度MPO-ANCA和高滴度抗组蛋白抗体。她接受了静脉内的类固醇治疗,随后在停用肼屈嗪后接受了免疫抑制治疗。临床快速好转后,该患者随后出院。结论。肼屈嗪诱导的抗中性粒细胞胞浆抗体阳性的肾血管炎是罕见的不良反应,可表现为严重的血管综合征,伴有多脏器受累。这种关联的特征包括存在高滴度的具有多重抗原性的抗髓过氧化物酶抗中性粒细胞胞质抗体,阳性抗组蛋白抗体,以及缺乏免疫球蛋白和补体沉积。立即停止肼屈嗪可能足以逆转疾病的活动,但可能需要进行免疫抑制。

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