Department of Medicine, Saint Agnes Hospital, Baltimore, MD, 21229, USA.
Section of Critical Care, Department of Medicine, Saint Agnes Hospital, Baltimore, MD, USA.
J Med Case Rep. 2020 Apr 15;14(1):47. doi: 10.1186/s13256-020-02378-w.
Hydralazine is a common vasodilator which has been used for the treatment of hypertension and heart failure. Hydralazine can induce antineutrophil cytoplasmic antibody-associated vasculitis due to its auto-immunogenic capability and one of the very rare presentations is pulmonary-renal syndrome.
We report a case of a 64-year-old African American woman, who presented to our emergency room with shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, fatigue, loss of appetite, cough with clear sputum, and lightheadedness. On admission, she developed acute hypoxic respiratory failure requiring intubation and acute renal failure requiring hemodialysis. A serologic workup was positive for antineutrophil cytoplasmic antibody, antinuclear antibody, anti-histone, anti-cardiolipin IgM, and anti-double-stranded DNA antibodies. A renal biopsy was done due to persistent deterioration in kidney function and demonstrated classic crescentic (pauci-immune) glomerulonephritis. Hydralazine was empirically discontinued early in the admission and she was started on corticosteroids and cyclophosphamide following biopsy results. She was clinically stable but remained dependent on hemodialysis after discharge.
Hydralazine-induced antineutrophil cytoplasmic antibody-associated vasculitis with pulmonary-renal syndrome is a rare occurrence. In the setting of hydralazine use, multiple positive antigens, and multisystem involvement, clinicians should consider this rare condition requiring prompt cessation of offending drug, early evaluation with biopsy, and contemplate empiric immunosuppressive therapy while biopsy confirmation is pending.
肼屈嗪是一种常用的血管扩张剂,用于治疗高血压和心力衰竭。肼屈嗪因其自身免疫原性而导致抗中性粒细胞胞质抗体相关性血管炎,其非常罕见的表现之一是肺-肾综合征。
我们报告了一例 64 岁非裔美国女性患者,因呼吸困难、端坐呼吸、阵发性夜间呼吸困难、腿部肿胀、疲劳、食欲不振、咳嗽伴白色痰和头晕而到我们的急诊室就诊。入院时,她发生急性低氧性呼吸衰竭,需要插管,急性肾衰竭需要血液透析。血清学检查抗中性粒细胞胞质抗体、抗核抗体、抗组蛋白、抗心磷脂 IgM 和抗双链 DNA 抗体阳性。由于肾功能持续恶化,进行了肾活检,结果显示为典型的新月体(寡免疫)肾小球肾炎。入院早期即停用肼屈嗪,并在活检结果后开始使用皮质类固醇和环磷酰胺。她的临床状况稳定,但出院后仍依赖血液透析。
肼屈嗪诱导的抗中性粒细胞胞质抗体相关性血管炎伴肺-肾综合征是一种罕见的情况。在使用肼屈嗪、多种阳性抗原和多系统受累的情况下,临床医生应考虑这种罕见的情况,需要立即停止使用致病药物,早期进行活检评估,并在等待活检确认的同时考虑经验性免疫抑制治疗。