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重叠自身免疫血清学的故事:诊断与孤立性肺部受累相关的肼屈嗪诱导的抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)

A Tale of Overlapping Autoimmune Serologies: Diagnosing Hydralazine-Induced Antineutrophilic Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV) Related With Isolated Pulmonary Involvement.

作者信息

Thapa Pratikshya, Yadav Indresh, Vinagolu-Baur Julia

机构信息

Department of Internal Medicine, Nuvance Health, Poughkeepsie, USA.

Department of Infectious Diseases, University of Louisville School of Medicine, Louisville, USA.

出版信息

Cureus. 2025 Aug 7;17(8):e89547. doi: 10.7759/cureus.89547. eCollection 2025 Aug.

Abstract

Hydralazine is an antihypertensive that can induce immune-related adverse effects, such as hydralazine-induced lupus and hydralazine-induced antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV). AAV involves necrotizing inflammation of small blood vessels, manifesting as fever, malaise, arthralgia, and myalgia, potentially leading to organ failure. Diagnosis includes clinical evaluation, serological testing for ANCA, and histopathological examination, confirmed by necrotizing granulomatous inflammation in affected tissues. This case report presents a rare presentation of hydralazine-induced AAV limited to the lungs, with isolated pleural involvement. A 57-year-old Caucasian male with hypertension, severe diastolic dysfunction, and end-stage renal disease undergoing dialysis presented with dyspnea, cough, and fatigue. Examination revealed bilateral lung crackles and peripheral edema. Chest computed tomography (CT) revealed a pleural effusion. Despite dialysis and thoracentesis, the patient showed no improvement, leading to pleural fluid analysis, which revealed an exudative pattern with reactive mesothelial cells. An autoimmune workup revealed elevated levels of antinuclear antibodies (ANA), anti-dsDNA, anti-histones, and ANCA. This presents a diagnostic dilemma: evolving systemic lupus erythematosus (SLE) or drug-induced vasculitis. Studies have highlighted that ANCA-positive patients may display serological features that overlap with SLE. Using the 2019 American College of Rheumatology (ACR) SLE criteria and biopsy results, we ruled out certain conditions and ultimately diagnosed the patient with chronic hydralazine-induced AAV. The patient's condition improved after hydralazine discontinuation and initiation of corticosteroid therapy. Pleural effusion can be an initial sign of AAV, and clinicians should be vigilant, especially in patients with end-stage renal disease who are unresponsive to dialysis.

摘要

肼屈嗪是一种抗高血压药物,可诱发免疫相关不良反应,如肼屈嗪诱发的狼疮和肼屈嗪诱发的抗中性粒细胞胞浆抗体(ANCA)相关血管炎(AAV)。AAV累及小血管的坏死性炎症,表现为发热、不适、关节痛和肌痛,可能导致器官衰竭。诊断包括临床评估、ANCA血清学检测和组织病理学检查,通过受累组织中的坏死性肉芽肿性炎症得以确诊。本病例报告呈现了一例罕见的肼屈嗪诱发的局限于肺部且仅有胸膜受累的AAV。一名57岁患有高血压、严重舒张功能障碍和正在接受透析的终末期肾病的白人男性,出现呼吸困难、咳嗽和疲劳。检查发现双侧肺部啰音和外周水肿。胸部计算机断层扫描(CT)显示胸腔积液。尽管进行了透析和胸腔穿刺术,但患者并无改善,遂进行胸腔积液分析,结果显示为渗出液且有反应性间皮细胞。自身免疫检查显示抗核抗体(ANA)、抗双链DNA、抗组蛋白和ANCA水平升高。这带来了一个诊断难题:是正在演变的系统性红斑狼疮(SLE)还是药物性血管炎。研究强调,ANCA阳性患者可能表现出与SLE重叠的血清学特征。根据2019年美国风湿病学会(ACR)SLE标准和活检结果,我们排除了某些情况,最终诊断该患者为慢性肼屈嗪诱发的AAV。停用肼屈嗪并开始使用皮质类固醇治疗后,患者病情有所改善。胸腔积液可能是AAV的初始表现,临床医生应保持警惕,尤其是对于对透析无反应的终末期肾病患者。

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