中血管炎

A. Abbasi Pérez , J. Campos Esteban , L. Antúnez Segura , E. Rico Sánchez-Mateos , E. Fernández Fernández
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引用次数: 0

摘要

中血管性血管炎是一种主要影响中肌和内脏动脉的炎症性疾病,可引起坏死性炎症。它们包括结节性多动脉炎(PAN)、川崎病(KD)和anca相关性血管炎(AAV)。PAN是一种少见的影响中型血管的血管炎。它的特点是全身性症状,如发烧、体重减轻和累及器官,如皮肤、肾脏和神经。它的诊断是基于组织学和影像学技术,显示特征性的动脉瘤。治疗包括皮质类固醇和免疫抑制剂。KD多见于儿童,累及冠状动脉,表现为持续发热、结膜炎、淋巴结病和皮肤粘膜病变。治疗包括静脉注射免疫球蛋白和乙酰水杨酸来预防动脉瘤。aav是坏死性血管炎,包括肉芽肿病合并多血管炎(GPA)、显微多血管炎(MPA)和嗜酸性肉芽肿病合并多血管炎(EGPA)。这些疾病影响中小血管,常累及肾脏、肺和上呼吸道。诊断基于ANCA阳性并经活检证实。治疗取决于严重程度,考虑到它可能危及生命,并将免疫抑制剂如利妥昔单抗或环磷酰胺与皮质类固醇联合使用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Vasculitis de mediano vaso
Medium-vessel vasculitides are inflammatory diseases that mainly affect medium muscle and visceral arteries, causing necrotizing inflammation. They include polyarteritis nodosa (PAN), Kawasaki disease (KD), and ANCA-associated vasculitis (AAV). PAN is a rare vasculitis that affects medium-sized vessels. It is characterized by systemic symptoms such as fever, weight loss, and involvement of organs such as the skin, kidneys, and nerves. Its diagnosis is based on histology and imaging techniques which show characteristic aneurysms. Treatment includes corticosteroids and immunosuppressants. KD, more common in children, affects coronary arteries and manifests as persistent fever, conjunctivitis, lymphadenopathy, and mucocutaneous lesions. Management includes intravenous immunoglobulins and acetylsalicylic acid to prevent aneurysms. AAVs are necrotizing vasculitides and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). These affect small- and medium-sized vessels, with frequent involvement of the kidneys, lungs, and upper respiratory tract. Diagnosis is based on ANCA positivity and confirmed by biopsy. Treatment depends on the severity, considering that it can be life-threatening, and combines immunosuppressants such as rituximab or cyclophosphamide with corticosteroids.
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