Erdheim-Chester disease, an uncommon non-Langerhans cell histiocytosis and its association with Sjögren's syndrome

Alejandra Espinosa Trujillo , Francisco Brañas Fernández , Aitor Abuín Blanco , Robustiano Pego Reigosa , María Rodríguez Rodríguez
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Abstract

Erdheim Chester disease (ECD) is a rare non-Langerhans cell histiocytosis. It is characterized by the infiltration of various organs and tissues by foamy histiocytes with a heterogeneous clinical course that varies from mild forms to disseminated forms with progressive and lethal behaviour. The case of a patient who presented with a cerebellar syndrome associated with autoimmune pathology is presented. In the course of the disease, refractoriness to glucocorticoids and clinical manifestation with bone involvement in the form of symmetrical osteosclerosis of long bones were determining factors for suspicion of this entity. We reviewed scientific articles through the PubMed metasearch engine with the keywords “erdheim chester disease”, “erdheim chester and nervous system”, and “autoimmunity and erdheim chester disease”, selecting those with greater emphasis on clinical presentation with neurological involvement and associated autoimmune pathology. Advances in the pathogenesis of ECD have allowed us to understand the nature of the disease, as well as the use of targeted therapies. It is interesting to keep this entity in mind, as well as the pathologies with which it is frequently associated, with the objective of an early diagnosis and a better clinical approach.
Erdheim-Chester病,一种罕见的非朗格汉斯细胞组织细胞增多症及其与Sjögren综合征的关系
Erdheim Chester病(ECD)是一种罕见的非朗格汉斯细胞组织细胞增多症。其特点是泡沫组织细胞浸润各种器官和组织,其临床病程不同,从轻度形式到弥散性形式,具有进行性和致死性行为。病例的病人谁提出了小脑综合征与自身免疫病理提出。在病程中,糖皮质激素的难治性和长骨对称性骨硬化累及骨骼的临床表现是怀疑该疾病的决定性因素。我们通过PubMed元搜索引擎,检索关键词为“埃尔德海姆切斯特病”、“埃尔德海姆切斯特病与神经系统”和“自身免疫与埃尔德海姆切斯特病”的科学文章,选择那些更强调神经系统受累和相关自身免疫病理的临床表现的文章。在ECD发病机制方面的进展使我们能够了解疾病的性质,以及靶向治疗的使用。有趣的是,要记住这个实体,以及与它经常相关的病理,目的是早期诊断和更好的临床方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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