panca/mpo (myeloperoxidase)-positive microscopic polyangiitis (mpa)

panca / mpo ( 髓过氧化物酶 ) 阳性的显微镜下多血管炎 ( mpa )
  • 文章类型: Case Reports
    我们描述了一名27岁男性并发嗜酸性肉芽肿合并多血管炎和混合性结缔组织疾病的罕见病例,肾,心脏,和皮肤表现。我们根据临床证实了诊断,组织病理学,和血清学标准。我们用皮质类固醇治疗了病人,甲氨蝶呤,环磷酰胺,和羟氯喹,实现早期缓解。同一患者中两种情况的共存极为罕见,并且仅在世界范围内的少数病例中报道。我们还回顾了关于这两种罕见的自身免疫性疾病共存的文献,发病机制,诊断,和管理。我们的案例强调识别具有复杂临床特征的患者的重叠自身免疫状况,并采用全面的诊断方法和量身定制的治疗策略。需要进一步的研究来了解这些患者的流行病学,预后,和最佳治疗。早期诊断和积极的免疫抑制对于实现缓解和预防器官损伤至关重要。我们还确定了该领域的知识差距和研究需求。
    We describe a rare case of concurrent eosinophilic granulomatosis with polyangiitis and mixed connective tissue disease in a 27-year-old man who presented with pulmonary, renal, cardiac, and skin manifestations. We confirmed the diagnosis based on clinical, histopathological, and serological criteria. We treated the patient with corticosteroids, methotrexate, cyclophosphamide, and hydroxychloroquine, achieving early remission. The coexistence of both conditions in the same patient is extremely rare and has only been reported in a few cases worldwide. We also review the literature on these two rare autoimmune diseases\' coexistence, pathogenesis, diagnosis, and management. Our case emphasizes recognizing overlapping autoimmune conditions in patients with complex clinical features and employing a comprehensive diagnostic approach and tailored treatment strategies. Further research is needed to understand these patients\' epidemiology, prognosis, and optimal therapy. Early diagnosis and aggressive immunosuppression are crucial for achieving remission and preventing organ damage. We also identified the knowledge gaps and research needs in this field.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    环磷酰胺(CYC)是一种免疫抑制药物,用于治疗各种风湿性疾病的危及生命的并发症,如血管炎和系统性红斑狼疮。这种药物的一种罕见的副作用是肺炎,发生在不到1%的患者中。我们描述了一个83岁的女性,有显微镜下多血管炎的病史,在休息时出现进行性呼吸困难,在劳累时加剧,并与CYC诱导的肺炎引起的端坐呼吸相关。在这次演讲前三个月,该患者被诊断为抗中性粒细胞胞浆抗体(ANCA)阳性的低免疫新月体和坏死性肾小球肾炎,并开始接受CYC治疗.一入场,计算机断层扫描(CT)胸部显示,呈马赛克分布的双侧磨玻璃影恶化,小叶间隔和小叶间隔增厚,未出现在三个月前进行的CT上。病人接受了广泛的检查,其中包括超声心动图,支气管镜与支气管肺泡灌洗,和病毒性呼吸道面板,以排除传染病和心脏病。她开始接受抗生素和利尿剂的经验性治疗,然而,尽管有这些干预措施,她继续呼吸窘迫。一个多学科小组召集,并对CYC诱导的肺损伤进行了诊断。CYC停止了,患者开始使用泼尼松,症状明显改善。该病例强调了认识到CYC是间质性肺炎的罕见原因的重要性。当考虑CYC诱导的肺毒性时,其他病因,例如机会性感染,心脏病因,弥漫性肺泡出血,应该排除。
    Cyclophosphamide (CYC) is an immunosuppressive medication used to treat life-threatening complications of various rheumatic diseases like vasculitis and systemic lupus erythematosus. A rare side effect of this medication is pneumonitis, which occurs in less than 1% of patients. We describe a case of an 83-year-old woman with a past medical history of microscopic polyangiitis, who presented with progressive dyspnea at rest, exacerbated on exertion, and associated with orthopnea that was attributed to CYC-induced pneumonitis. Three months before this presentation, the patient was diagnosed with antineutrophil cytoplasmic antibodies (ANCA)-positive pauci-immune crescentic and necrotizing glomerulonephritis and started on CYC. On admission, a computed tomography (CT) chest showed worsening bilateral ground-glass opacities in a mosaic distribution and inter and intralobular septal thickening, not present on the CT performed three months prior. The patient underwent an extensive workup, which included an echocardiogram, bronchoscopy with bronchoalveolar lavage, and viral respiratory panel to rule out infectious and cardiac pathologies. She was started on empiric treatment with antibiotics and diuretics, however, despite these interventions, she continued with respiratory distress. A multidisciplinary team convened, and the diagnosis of CYC-induced lung injury was entertained. The CYC was discontinued, and the patient was started on prednisone with significant improvement in symptoms. This case highlights the importance of recognizing CYC as a rare cause of interstitial pneumonitis. When considering CYC-induced lung toxicity, other etiologies, such as opportunistic infections, cardiac etiologies, and diffuse alveolar hemorrhage, should be ruled out.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

公众号