Castillo Diego, Martin-Arroyo Iñigo, Moreno Amalia, Balcells Eva, Villar Ana, Vicens-Zygmunt Vanesa, Vendrell Montserrat, Belda Sonia, Portillo Karina, Kettou Dalila, Aparicio Francisco, Molina-Molina Maria
Servicio de Neumología, Hospital de la Santa Creu i Sant Pau, Institut d'Investigacions Biomèdiques (IIB-Sant Pau), Barcelona, España.
Servicio de Neumología, Hospital de la Santa Creu i Sant Pau, Institut d'Investigacions Biomèdiques (IIB-Sant Pau), Barcelona, España.
Med Clin (Barc). 2014 Nov 18;143(10):433-9. doi: 10.1016/j.medcli.2013.11.039. Epub 2014 Apr 13.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare respiratory disease closely associated with smoking. The aim of the study is to know the reality of PLCH in the Catalonian population.
We included all patients diagnosed of PLCH in 8 different Catalonian hospitals from April 2011 to December 2012. Epidemiologic, clinical, radiological, functional, diagnostic and therapeutic variables were collected, both at the beginning and at the last visit of the process.
Thirty two patients were included. Overall, patients were middle-aged (38 years), with no gender predominance. Most of them were smokers (71.9%). The main pulmonary function values (forced vital capacity 75.9%, forced expiratory volume in one second 68.9%, diffusing capacity of the lung for carbon monoxide 59.6%) and radiological findings (cysts 89.7%, nodules 60.3%) showed no significant changes during evolution. However, the disease progressed with a worsening lung function in 34.4% of cases. The analysis of the functional outcome (forced vital capacity, forced expiratory volume in one second, and diffusing capacity of the lung for carbon monoxide) did not reveal significant differences according to the persistence or not of smoking (P<0.05).
Our patients with PLCH had similar clinical and radiological characteristics to those described in other populations. The results observed regarding the relationship with smoking suggest that there may be other factors beyond smoking, which influence on the evolution of the disease. It therefore seems advisable to design prospective studies to investigate this aspect.
肺朗格汉斯细胞组织细胞增多症（PLCH）是一种与吸烟密切相关的罕见呼吸系统疾病。本研究旨在了解加泰罗尼亚人群中PLCH的实际情况。
我们纳入了2011年4月至2012年12月期间在8家不同的加泰罗尼亚医院被诊断为PLCH的所有患者。收集了疾病过程开始时和最后一次就诊时的流行病学、临床、放射学、功能、诊断和治疗变量。
共纳入32例患者。总体而言，患者为中年（38岁），无性别优势。他们中的大多数是吸烟者（71.9%）。主要肺功能指标（用力肺活量75.9%、一秒用力呼气量68.9%、肺一氧化碳弥散量59.6%）和放射学表现（囊肿89.7%、结节60.3%）在疾病进展过程中无显著变化。然而，34.4%的病例中疾病进展伴有肺功能恶化。根据是否持续吸烟对功能结局（用力肺活量、一秒用力呼气量和肺一氧化碳弥散量）进行分析，未发现显著差异（P<0.05）。
我们的PLCH患者在临床和放射学特征上与其他人群中描述的相似。关于与吸烟关系的观察结果表明，除吸烟外可能还有其他因素影响疾病的进展。因此，设计前瞻性研究来调查这方面似乎是可取的。
