Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, INSERM U1016, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
Department of Cardiology and Heart Transplantation, Ospedale Niguarda, Milan, Italy.
J Heart Lung Transplant. 2014 Aug;33(8):842-50. doi: 10.1016/j.healun.2014.02.023. Epub 2014 Feb 24.
Heart involvement is the leading cause of death of patients with eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) and is more frequent in anti-neutrophil cytoplasm antibody (ANCA)-negative patients. Post-transplant outcome has only been reported once.
We conducted a retrospective international multicenter study. Patients satisfying the criteria of the American College of Rheumatology and/or revised Chapel Hill Consensus Conference Nomenclature were identified by collaborating vasculitis and transplant specialists, and the help of the Churg-Strauss Syndrome Association.
Nine ANCA(-) patients who received transplants between October 1987 and December 2009 were identified. The vasculitis and cardiomyopathy diagnoses were concomitant for 5 patients and separated by 12 to 288 months for the remaining 4 patients. Despite ongoing immunosuppression, histologic examination of 7 (78%) patients' explanted hearts showed histologic patterns suggestive of active vasculitis. The overall 5-year survival rate was low (57%), but rose to 80% when considering only the 6 patients transplanted during the last decade. After survival lasting 3 to 60 months, 4 (44%) patients died sudden deaths.
The search for EGPA-related cardiomyopathy is mandatory early in the course of this type of vasculitis. Indeed, prompt treatment with corticosteroids and cyclophosphamide may achieve restore cardiac function. Most patients in this series were undertreated. For patients with refractory EGPA, heart transplantation should be performed, which carries a fair prognosis. No optimal immunosuppressive strategy has yet been identified.
心脏受累是嗜酸性肉芽肿性多血管炎（EGPA；以前称为 Churg-Strauss 综合征）患者死亡的主要原因，在抗中性粒细胞胞质抗体（ANCA）阴性患者中更为常见。移植后的结果仅报告过一次。
我们进行了一项回顾性国际多中心研究。通过合作的血管炎和移植专家以及 Churg-Strauss 综合征协会的帮助，确定了符合美国风湿病学会和/或修订后的 Chapel Hill 共识会议命名标准的患者。
确定了 9 名于 1987 年 10 月至 2009 年 12 月期间接受移植的 ANCA（-）患者。5 名患者的血管炎和心肌病诊断同时发生，其余 4 名患者的间隔时间为 12 至 288 个月。尽管持续进行免疫抑制治疗，但对 7 名（78%）患者的移植心脏进行组织学检查显示出提示活动性血管炎的组织学模式。总体 5 年生存率较低（57%），但当仅考虑过去十年期间接受移植的 6 名患者时，生存率上升至 80%。在存活 3 至 60 个月后，4 名（44%）患者突然死亡。
在这种类型的血管炎早期，必须寻找 EGPA 相关的心肌病。实际上，及时用皮质类固醇和环磷酰胺治疗可能会恢复心脏功能。该系列中的大多数患者治疗不足。对于难治性 EGPA 患者，应进行心脏移植，这具有良好的预后。尚未确定最佳的免疫抑制策略。
