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儿童系统性血管炎的肺部表现。

Pulmonary Manifestations of Systemic Vasculitis in Children.

机构信息

Department of Pediatric Rheumatology, Hacettepe University, Sihhiye Campus, Ankara 06100, Turkey.

Department of Pediatric Rheumatology, Hacettepe University, Sihhiye Campus, Ankara 06100, Turkey.

出版信息

Pediatr Clin North Am. 2021 Feb;68(1):167-176. doi: 10.1016/j.pcl.2020.09.014.

Abstract

Vasculitides are defined according to the vessel size involved, and they tend to affect certain organ systems. Pulmonary involvement is rare in the common childhood vasculitides, such as Kawasaki disease, IgA vasculitis (Henoch Schonlein purpura). On the other hand, lung involvement is common in a rare pediatric vasculitis, granulomatosis with polyangiitis (GPA) (Wegener granulomatosis), where respiratory system findings are common. A criterion in the Ankara 2008 classification criteria for GPA is the presence of nodules, cavities, or fixed infiltrates. The adult data suggest that rituximab may be an alternative to cyclophosphamide in induction treatment.

摘要

血管炎根据受累血管的大小来定义,它们往往影响某些器官系统。在常见的儿童血管炎中,如川崎病、IgA 血管炎(过敏性紫癜),肺部受累很少见。另一方面,在罕见的儿童血管炎,如肉芽肿伴多血管炎(GPA)(韦格纳肉芽肿),肺部受累很常见,呼吸系统表现很常见。2008 年安卡拉 GPA 分类标准中的一个标准是存在结节、空洞或固定浸润。成人数据表明,利妥昔单抗可能是环磷酰胺诱导治疗的替代药物。

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