Department of Surgery, College of Medicine, University of Central Florida, Orlando, Florida; and.
Department of Surgery, College of Medicine, University of Central Florida, Orlando, Florida; and
Pediatrics. 2021 Jan;147(1). doi: 10.1542/peds.2020-0932.
Granulomatosis with polyangiitis is an antineutrophil cytoplasmic antibody-associated vasculitis that primarily affects small vessels. The disease typically affects the respiratory tract and kidneys but has also been known to involve the gastrointestinal tract, genitourinary tract, thyroid, and liver. Cardiac involvement is rare. Coronary artery aneurysms (CAAs) are an extremely uncommon finding, with only 1 reported case in an adult patient and no pediatric cases reported to date. Here, we report the unique case of a child with granulomatosis with polyangiitis who initially presented with fever of unknown origin and pulmonary and renal symptoms with no cardiac complaints. An echocardiogram revealed severe bilateral fusiform CAAs. Because of the high risk of mortality posed by the severity of her renal and cardiac disease, the patient was managed with intensive induction immunosuppression with steroids, rituximab, and cyclophosphamide. She is maintained on steroids, rituximab, aspirin, and warfarin with improved renal function but no change in her CAAs.
肉芽肿性多血管炎是一种抗中性粒细胞胞浆抗体相关性血管炎，主要影响小血管。这种疾病通常影响呼吸道和肾脏，但也有胃肠道、泌尿生殖道、甲状腺和肝脏受累的报道。心脏受累很少见。冠状动脉瘤（CAA）是一种非常罕见的发现，在成年患者中仅报告过 1 例，迄今为止尚未报告儿科病例。在这里，我们报告了一例儿童肉芽肿性多血管炎的独特病例，该患儿最初表现为不明原因发热和肺部及肾脏症状，无心脏主诉。超声心动图显示严重的双侧梭形 CAA。由于她的肾脏和心脏疾病的严重程度导致死亡率很高，该患者接受了强化诱导免疫抑制治疗，包括类固醇、利妥昔单抗和环磷酰胺。她目前仍在接受类固醇、利妥昔单抗、阿司匹林和华法林治疗，肾功能有所改善，但 CAA 没有变化。
