多动脉炎重叠综合征:结节性多动脉炎伴白细胞分裂性血管炎伴左室血栓和声带麻痹1例报告。

Q2 Medicine Oman Medical Journal Pub Date : 2024-07-31 eCollection Date: 2024-07-01 DOI:10.5001/omj.2024.25
Wahinuddin Sulaiman, Henry Foong Boon Bee, Lim Wei Mei, Ng Theng Chun, Chin Yow Wen, Lee Bang Rom, Syed Ibrahim Jamalullail
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引用次数: 0

摘要

多血管炎重叠综合征(POS)是一种系统性血管炎,其特征是多个明确的血管综合征的重叠特征。我们提出的情况下,38岁的马来男子进行性呼吸困难和可触及紫癜在他的下肢。诊断结果显示右侧肺节段性实变伴胸腔积液,心内血栓伴收缩期心功能障碍,以及继发于喉单神经病变的左侧声带麻痹。抗中性粒细胞细胞质抗体检测结果为阴性,皮肤活检显示白细胞破裂性血管炎(LCV),免疫荧光研究为阴性。患者符合美国风湿病学会结节性多动脉炎和特发性LCV的诊断标准。医学文献中仅报道了3例POS合并结节性多动脉炎与特发性LCV重叠的病例。据我们所知,这是首次报道的POS与心肺累及和喉单神经病变。该患者对使用高剂量皮质类固醇和霉酚酸酯的缓解诱导疗法有极好的反应,并在接受类固醇逐渐减少治疗时仍处于缓解期。及时开始治疗对于预防血管并发症和不可逆的器官功能障碍至关重要。在这种情况下,霉酚酸酯可作为环磷酰胺的可接受替代品作为缓解诱导剂。
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Polyangiitis Overlap Syndrome: Polyarteritis Nodosa with Leukocytoclastic Vasculitis Associated with Left Ventricular Thrombus and Vocal Cord Palsy: A Case Report.

Polyangiitis overlap syndrome (POS) is a systemic vasculitis characterized by overlapping features of more than one well-defined vasculitic syndrome. We present the case of a 38-year-old Malay man with progressive dyspnea and palpable purpura in his lower limbs. The diagnostic evaluation revealed right-sided segmental pulmonary consolidation with pleural effusion, systolic cardiac dysfunction with the presence of an intracardiac thrombus, and left vocal cord palsy secondary to laryngeal mononeuropathy. Anti-neutrophil cytoplasmic antibodies testing yielded negative results, and skin biopsy revealed leukocytoclastic vasculitis (LCV) with negative immunofluorescence studies. The patient fulfilled the American College of Rheumatology diagnostic criteria for polyarteritis nodosa and idiopathic LCV. Only three case reports of POS with a combination of polyarteritis nodosa overlapping with idiopathic LCV have been reported in the medical literature. To the best of our knowledge, this is the first report of POS with cardiopulmonary involvement and laryngeal mononeuropathy. This patient had an excellent response to remission induction therapy using high-dose corticosteroids and mycophenolate mofetil and remains in remission while undergoing a steroid taper. Timely initiation of treatment is essential to prevent vasculitic complications and irreversible organ dysfunction. Mycophenolate mofetil may serve as an acceptable alternative to cyclophosphamide as a remission induction agent in this condition.

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来源期刊
Oman Medical Journal
Oman Medical Journal Medicine-Medicine (all)
CiteScore
3.10
自引率
0.00%
发文量
119
审稿时长
12 weeks
期刊最新文献
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