Abstract：
Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis with diverse clinical features. It is characterized by systemic histiocyte infiltration of the bone, skin, central nervous system, lung, kidney, and cardiovascular system. Pericardial involvement is frequently revealed through either pericardial effusion or pericardial thickening in patients with ECD. Although most patients remain asymptomatic, progressive pericarditis, effusion, or cardiac tamponade may occur. Herein, we report a rare and unusual presentation of ECD in a 51-year-old man who experienced severe constrictive pericarditis. The patient presented with uncontrolled fluid retention and heart failure. After the diagnosis of ECD, interferon alpha treatment was administered. The patient recovered dramatically with decreased pleural and pericardial effusion, as well as improvements in the echocardiographic signs of constrictive pericarditis. Despite several therapeutic options described in the literature for managing ECD-related pericardial disease, a standard treatment has not been established. This report highlights the importance of early treatment based on accurate diagnosis of an unusual ECD complication.
摘要：
Erdheim-Chester病（ECD）是一种罕见的疾病，具有不同临床特征的非朗格汉斯细胞组织细胞增生症。它的特征是骨骼的全身组织细胞浸润，皮肤,中枢神经系统,肺，肾,和心血管系统。在ECD患者中，通常通过心包积液或心包增厚来揭示心包受累。尽管大多数患者仍然无症状，进行性心包炎,积液,或者可能发生心脏填塞。在这里，我们报道了一例发生严重缩窄性心包炎的51岁男性患者罕见且不寻常的ECD表现.患者出现不受控制的液体潴留和心力衰竭。ECD诊断后，给予干扰素α治疗。患者恢复明显，胸膜和心包积液减少，以及缩窄性心包炎的超声心动图征象的改善。尽管文献中描述了几种治疗ECD相关心包疾病的治疗选择，尚未建立标准治疗方法。该报告强调了基于对异常ECD并发症的准确诊断的早期治疗的重要性。
