Akkurt Esma Sevil, Akinci Ozyurek Berna, Ensarioglu Kerem, Sahin Ozdemirel Tugce, Zenbilli Esma, Erturk Hakan
Department of Chest Disease, Atatürk Sanatorium Research and Training Hospital, University of Health Sciences, Ankara, Turkey.
Department of Radiology, Atatürk Sanatorium Research and Training Hospital, University of Health Sciences, Ankara, Turkey.
Medicine (Baltimore). 2025 Aug 8;104(32):e43766. doi: 10.1097/MD.0000000000043766.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease of unknown etiology, typically affecting individuals aged 20 to 40. Characteristic high-resolution computed tomography findings may obviate the need for biopsy. This study aims to raise awareness by describing clinical and imaging features of PLCH. This single-center retrospective cohort (2016-2024) included 26 confirmed PLCH patients. Demographics, smoking history, comorbidities, symptoms, lab results, 6-minute walk distance, pulmonary function tests, diffusion capacity for carbon monoxide (DLCO), and high-resolution computed tomography findings were reviewed. Median age was 46 years; 84.6% had a smoking history. Cough (46.2%) and dyspnea (53.8%) were common. Radiologically, cysts and nodules predominated. Initial DLCO was lower in dyspneic patients (P = .035). Over median 2.5 years of follow-up, pulmonary function tests and DLCO values remained stable. Early recognition of PLCH radiological features and smoking cessation are crucial. Despite limitations, these findings reinforce known patterns and highlight the need for larger, prospective studies.
肺朗格汉斯细胞组织细胞增多症（PLCH）是一种病因不明的罕见间质性肺病，通常影响20至40岁的个体。高分辨率计算机断层扫描的特征性表现可能无需进行活检。本研究旨在通过描述PLCH的临床和影像学特征来提高认识。这项单中心回顾性队列研究（2016 - 2024年）纳入了26例确诊的PLCH患者。回顾了人口统计学、吸烟史、合并症、症状、实验室检查结果、6分钟步行距离、肺功能测试、一氧化碳弥散量（DLCO）和高分辨率计算机断层扫描结果。中位年龄为46岁；84.6%有吸烟史。咳嗽（46.2%）和呼吸困难（53.8%）很常见。放射学上，以囊肿和结节为主。呼吸困难患者的初始DLCO较低（P = 0.035）。在中位2.5年的随访中，肺功能测试和DLCO值保持稳定。早期识别PLCH的放射学特征和戒烟至关重要。尽管存在局限性，但这些发现强化了已知模式，并突出了进行更大规模前瞻性研究的必要性。
