Medical College of Wisconsin, Children's Hospital of Wisconsin, Milwaukee, WI, USA.
Pediatr Rheumatol Online J. 2012 Apr 13;10(1):9. doi: 10.1186/1546-0096-10-9.
Granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) is a systemic necrotizing vasculitis of unknown etiology that commonly involves the upper airways, lungs, and kidneys. Cardiac involvement with an intracardiac mass is an exceedingly rare manifestation of this disease, especially in the pediatric population where, to our knowledge, only one article exists to date that has described such a finding. In this report, we present the case of an adolescent female who initially presented with renal failure and an intracardiac mass. Subsequent work-up led to a diagnosis of granulomatosis with polyangiitis (GPA). Cardiac manifestations in pediatric GPA are not common; however, they may be more prevalent than reported given recent adult literature and concern for clinically silent abnormalities.
肉芽肿性多血管炎(GPA;韦格纳肉芽肿病)是一种病因不明的系统性坏死性血管炎,常累及上呼吸道、肺和肾脏。心脏受累并伴有心内肿块是这种疾病极罕见的表现,尤其是在儿科人群中,据我们所知,迄今为止只有一篇文章描述了这种发现。在本报告中,我们介绍了一位最初表现为肾衰竭和心内肿块的青春期女性患者。进一步的检查导致诊断为肉芽肿性多血管炎(GPA)。儿科 GPA 的心脏表现并不常见;然而,鉴于最近的成人文献和对临床无症状异常的关注,它们的患病率可能比报告的要高。