PDF(Pubmed)
Abstract：
UNASSIGNED: Erdheim-Chester disease (ECD) is a rare multisystem disorder that primarily affects adults. It is characterized by the excessive production and accumulation of histiocytes, a type of white blood cell, within multiple tissues and organs, including the cardiovascular system. The infiltration of histiocytes can cause a range of cardiovascular symptoms, including pericardial effusion, myocardial infiltration, and heart failure, among others. Despite the potential severity of these cardiovascular manifestations, ECD is often misdiagnosed or underdiagnosed, leading to delays in appropriate treatment and poor outcomes for patients. As such, there is a pressing need for increased awareness and understanding of ECD\'s cardiovascular manifestations among clinicians and researchers. This article aims to highlight the importance of considering ECD as a potential underlying cause of cardiovascular complaints and to encourage further investigation into this uncommon but potentially life-threatening condition.
UNASSIGNED: A 63-year-old man presented as outpatient complaining of dyspnoea on exertion during the last 3 weeks (New York Heart Association functional class III). He had also experienced a left shoulder and bilateral knee pain over the last 6 months. The patient was found to have a massive pericardial effusion associated with ECD. While pericardial effusions can have various causes, including infection, cancer, and autoimmune disorders, ECD is one potential cause of this condition. Therefore, it is important for clinicians to consider ECD in the differential diagnosis of patients presenting with unexplained pericardial effusions, particularly in the context of other systemic symptoms suggestive of ECD. We discuss about this specific aetiology and the clinical management of this uncommon condition.
UNASSIGNED: Erdheim-Chester disease, a non-Langerhans cell histiocytosis, is a rare multisystem disorder. Diagnosis is challenging and should be suspected in the presence of a pericardial effusion with conduction abnormalities with indicators of a multisystem disease.
UNASSIGNED: A 63-year-old man presented as outpatient complaining of dyspnoea on exertion during the last 3 weeks (New York Heart Association functional class III). He had also experienced a left shoulder and bilateral knee pain over the last 6 months. The patient was found to have a massive pericardial effusion associated with ECD. While pericardial effusions can have various causes, including infection, cancer, and autoimmune disorders, ECD is one potential cause of this condition. Therefore, it is important for clinicians to consider ECD in the differential diagnosis of patients presenting with unexplained pericardial effusions, particularly in the context of other systemic symptoms suggestive of ECD. We discuss about this specific aetiology and the clinical management of this uncommon condition.
UNASSIGNED: Erdheim-Chester disease, a non-Langerhans cell histiocytosis, is a rare multisystem disorder. Diagnosis is challenging and should be suspected in the presence of a pericardial effusion with conduction abnormalities with indicators of a multisystem disease.
摘要：
Erdheim-Chester病（ECD）是一种罕见的多系统疾病，主要影响成人。它的特点是组织细胞的过度产生和积累，一种白细胞,在多个组织和器官中，包括心血管系统.组织细胞的浸润会引起一系列的心血管症状,包括心包积液,心肌浸润,心力衰竭,在其他人中。尽管这些心血管表现的潜在严重程度，ECD常被误诊或漏诊,导致适当治疗的延误和患者的不良预后。因此，临床医生和研究人员迫切需要提高对ECD心血管表现的认识和理解.本文旨在强调将ECD视为心血管疾病的潜在根本原因的重要性，并鼓励对这种罕见但可能危及生命的疾病进行进一步调查。
■一名63岁的男子在过去3周内因劳累而出现呼吸困难（纽约心脏协会功能等级III）。在过去的6个月中，他还经历了左肩和双侧膝盖疼痛。发现患者有大量心包积液与ECD相关。虽然心包积液有多种原因，包括感染,癌症,和自身免疫性疾病，ECD是这种情况的一个潜在原因。因此,临床医生在对不明原因心包积液患者的鉴别诊断中考虑ECD是非常重要的,特别是在其他提示ECD的全身症状的情况下。我们讨论了这种特殊的病因以及这种罕见疾病的临床处理。
■Erdheim-Chester病,非朗格汉斯细胞组织细胞增生症，是一种罕见的多系统疾病。诊断具有挑战性，应在存在传导异常的心包积液并具有多系统疾病指标的情况下进行怀疑。
■一名63岁的男子在过去3周内因劳累而出现呼吸困难（纽约心脏协会功能等级III）。在过去的6个月中，他还经历了左肩和双侧膝盖疼痛。发现患者有大量心包积液与ECD相关。虽然心包积液有多种原因，包括感染,癌症,和自身免疫性疾病，ECD是这种情况的一个潜在原因。因此,临床医生在对不明原因心包积液患者的鉴别诊断中考虑ECD是非常重要的,特别是在其他提示ECD的全身症状的情况下。我们讨论了这种特殊的病因以及这种罕见疾病的临床处理。
■Erdheim-Chester病,非朗格汉斯细胞组织细胞增生症，是一种罕见的多系统疾病。诊断具有挑战性，应在存在传导异常的心包积液并具有多系统疾病指标的情况下进行怀疑。
