Respiratory Department, Musgrove Park Hospital, Taunton, Somerset, UK.
Respiratory Department, Royal United Hospital, Bath, UK.
Thorax. 2014 Aug;69(8):766-7. doi: 10.1136/thoraxjnl-2013-204313. Epub 2014 Jan 30.
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease of unknown aetiology. We aimed to characterise a UK-wide cohort of patients with PLCH and compare diagnostic and management methods in specialist and non-specialist centres. 106 cases (53 hospitals) identified. Complete data received in 67 cases (53.7% female, age 37.1±14.4 years). 96% current or ex-smokers. Treatment; smoking cessation (79%), corticosteroids (30.6%), cytotoxic therapy (26.9%) and lung transplant (6%). Patients at specialist centres received cytotoxic drugs more often (p=0.0001) and survival appeared higher. This dataset indicates a more even gender distribution than previously documented. It suggests variation in clinical management and outcomes achieved dependent on clinical experience.
肺朗格汉斯细胞组织细胞增生症（PLCH）是一种病因不明的罕见间质性肺病。我们旨在描述英国范围内 PLCH 患者的队列，并比较专科和非专科中心的诊断和治疗方法。共确定了 106 例病例（53 家医院）。在 67 例病例中（53.7%为女性，年龄 37.1±14.4 岁）收到了完整的数据。96%为现吸烟者或曾吸烟者。治疗方法：戒烟（79%）、皮质类固醇（30.6%）、细胞毒性药物治疗（26.9%）和肺移植（6%）。在专科中心的患者更常接受细胞毒性药物治疗（p=0.0001），且生存率似乎更高。该数据集表明，与之前记录的相比，该病的性别分布更加均衡。这表明临床管理和治疗结果存在差异，取决于临床经验。
