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多血管炎重叠综合征:结节性多动脉炎合并白细胞破碎性血管炎伴左心室血栓形成和声带麻痹:病例报告

Polyangiitis Overlap Syndrome: Polyarteritis Nodosa with Leukocytoclastic Vasculitis Associated with Left Ventricular Thrombus and Vocal Cord Palsy: A Case Report.

作者信息

Sulaiman Wahinuddin, Bee Henry Foong Boon, Mei Lim Wei, Chun Ng Theng, Wen Chin Yow, Rom Lee Bang, Jamalullail Syed Ibrahim

机构信息

Department of Medicine, Faculty of Medicine, Royal College of Medicine, Universiti Kuala Lumpur, Perak, Malaysia.

Department of Medicine, KPJ Ipoh Specialist Hospital, Perak, Malaysia.

出版信息

Oman Med J. 2024 Jul 31;39(4):e659. doi: 10.5001/omj.2024.25. eCollection 2024 Jul.

Abstract

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.

摘要

多血管炎重叠综合征(POS)是一种系统性血管炎,其特征为具有不止一种明确的血管炎综合征的重叠特征。我们报告一例38岁马来男性病例,该患者有进行性呼吸困难且下肢可触及紫癜。诊断评估显示右侧节段性肺实变伴胸腔积液、收缩性心功能不全伴心内血栓形成,以及继发于喉单神经病的左侧声带麻痹。抗中性粒细胞胞浆抗体检测结果为阴性,皮肤活检显示白细胞破碎性血管炎(LCV)且免疫荧光研究为阴性。该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。医学文献中仅报道过3例结节性多动脉炎与特发性LCV重叠的POS病例报告。据我们所知,这是首例伴有心肺受累和喉单神经病的POS报告。该患者对使用大剂量糖皮质激素和霉酚酸酯的缓解诱导治疗反应良好,在接受糖皮质激素减量时仍处于缓解状态。及时开始治疗对于预防血管炎并发症和不可逆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作为缓解诱导剂的可接受替代药物。

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