PDF(Pubmed)
Abstract：
Erdheim-Chester Disease (ECD) is a rare form of histiocytosis characterized by xanthomatous infiltration of affected organs. We present a case of a 62-year-old man with ECD initially presenting with constrictive pericarditis. Comprehensive imaging revealed systemic involvement, including the skeleton, orbit, pituitary, lung, kidney, and retroperitoneum, despite the absence of related symptoms. The diagnosis of ECD was eventually confirmed through histopathological evidence from a CT-guided biopsy. The patient responded well to interferon-α2b treatment, with gradual symptom amelioration and improvement in imaging and laboratory findings over a 5-month follow-up period. This case highlights the importance of considering ECD in the differential diagnosis of constrictive pericarditis and the utility of multimodal imaging for accurate diagnosis and management of this rare disease. The patient\'s positive response to treatment also highlights the potential for effective management of ECD, particularly with early diagnosis and intervention.
摘要：
Erdheim-Chester病（ECD）是一种罕见的组织细胞增生症，其特征是受影响器官的黄瘤浸润。我们介绍了一例62岁的ECD患者，最初表现为缩窄性心包炎。综合影像学显示全身受累，包括骷髅,轨道,垂体,肺，肾,和腹膜后,尽管没有相关症状。通过CT引导活检的组织病理学证据最终证实了ECD的诊断。患者对干扰素-α2b治疗反应良好，在5个月的随访期内，症状逐渐改善，影像学和实验室检查结果也有所改善。该病例强调了在缩窄性心包炎的鉴别诊断中考虑ECD的重要性，以及多模态成像对这种罕见疾病的准确诊断和治疗的实用性。患者对治疗的积极反应也突出了有效管理ECD的潜力，特别是早期诊断和干预。
