关键词: antineutrophil cytoplasmic antibody (anca) associated vasculitis (aav) churg-strauss syndrome dermatopathology eosinophilic granulomatosis with polyangiitis (egpa) granulomatosis with polyangiitis (gpa) panca/mpo (myeloperoxidase)-positive microscopic polyangiitis (mpa) polymyositis sle pathogenesis systemic lupus erythematosus vasculitis

来  源:   DOI:10.7759/cureus.43584   PDF(Pubmed)

Abstract:
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.
摘要:
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。
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