Zhu Dan, Luo Yiming, Liu Xiangyuan, Zu Lingyun
Department of Cardiology, Peking University Third Hospital, Key Laboratory of Cardiovascular Molecular Biology and Regulatory Peptides, Ministry of Health, Beijing, China.
Department of Medicine, Mount Sinai St. Luke's and Mount Sinai West Hospitals, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Case Rep Rheumatol. 2017;2017:2908185. doi: 10.1155/2017/2908185. Epub 2017 Jan 29.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis commonly with cardiac complications. We describe a case of anti-PR3 ANCA-positive EGPA complicated by congestive heart failure and intraventricular thrombosis. Interestingly, the thrombus was resolved rapidly with steroid and cyclophosphamide in the setting of interrupted anticoagulation. To the best of our knowledge, we report the first case of anti-PR3 positive EGPA with extensive cardiac involvement. Our patient had overlapping features with previously studied ANCA-positive and ANCA-negative EGPA cases. We also hypothesize that the thrombogenic potential of eosinophils may play a central role in thrombogenesis in EGPA and aggressive immunosuppressive therapy remains the cornerstone of treatment, and the addition of anticoagulation therapy in the setting of thrombus formation and also very high risk of bleeding needs to be considered cautiously.
嗜酸性肉芽肿性多血管炎（EGPA）是一种罕见的系统性血管炎，常伴有心脏并发症。我们描述了一例抗PR3 ANCA阳性的EGPA，并发充血性心力衰竭和心室内血栓形成。有趣的是，在中断抗凝的情况下，使用类固醇和环磷酰胺后血栓迅速溶解。据我们所知，我们报告了首例抗PR3阳性且有广泛心脏受累的EGPA病例。我们的患者具有与先前研究的ANCA阳性和ANCA阴性EGPA病例重叠的特征。我们还推测，嗜酸性粒细胞的致血栓形成潜力可能在EGPA的血栓形成中起核心作用，积极的免疫抑制治疗仍然是治疗的基石，在血栓形成且出血风险非常高的情况下，是否添加抗凝治疗需要谨慎考虑。
