Carbone Roberto G, Roden Anja C, Huntley Christopher C, Puppo Francesco
Department of Internal Medicine, University of Genoa, Genoa, Italy.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
J Clin Imaging Sci. 2025 Jun 10;15:21. doi: 10.25259/JCIS_42_2025. eCollection 2025.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare diffuse parenchymal lung disease representing 3% of all interstitial lung diseases. PLCH occurs either as part of multi-system Langerhans cell histiocytosis (LCH) or an isolated disease of the lung. Isolated involvement of lungs is observed in approximately 50-70% of LCH cases and the age of onset peaks between 20 and 40 years without gender difference. Cigarette smoking is the only identified environmental risk factor. PLCH is characterized by Langerhans cell infiltrates that in general are in a bronchiolocentric distribution. Pulmonary lesions can occur after chronic systemic disease or are an initial sign of disease. The aims of this study are to (1) report five rare cases of PLCH in adults; (2) discuss the relationship of PLCH with interstitial lung fibrosis; (3) provide a concise review of PLCH epidemiology, pathophysiology, diagnosis, treatment, and prognosis to improve the knowledge of PLCH by pneumologists and internal medicine physicians.
肺朗格汉斯细胞组织细胞增多症（PLCH）是一种罕见的弥漫性实质性肺疾病，占所有间质性肺疾病的3%。PLCH可作为多系统朗格汉斯细胞组织细胞增多症（LCH）的一部分出现，也可为孤立的肺部疾病。在大约50%-70%的LCH病例中观察到肺部孤立受累，发病年龄高峰在20至40岁之间，无性别差异。吸烟是唯一已确定的环境危险因素。PLCH的特征是朗格汉斯细胞浸润，通常呈细支气管中心分布。肺部病变可发生在慢性全身性疾病之后，或是疾病的初始表现。本研究的目的是：（1）报告5例成人罕见的PLCH病例；（2）讨论PLCH与间质性肺纤维化的关系；（3）简要综述PLCH的流行病学、病理生理学、诊断、治疗和预后，以提高肺科医生和内科医生对PLCH的认识。
