Pulmonary Langerhans cell histiocytosis (PLCH): a new UK register. Issue 8 (30th January 2014)

• Record Type: Journal Article
• Authors: Mason, Rebecca H; Foley, Noeleen M; Branley, Howard M; Adamali, Huzaifa I; Hetzel, Martin; Maher, Toby M; Suntharalingam, Jay
• Is Part Of: Thorax. Volume 69:Issue 8(2014)
• Page Start: 766
• Page End: 767
• Publication Date: 2014-01-30
• Subjects: Rare lung diseases -- Interstitial Fibrosis; Chest -- Diseases -- Periodicals; Thorax; Chest -- Diseases; Periodicals; Periodicals; 617.54
• DOI: 10.1136/thoraxjnl-2013-204313
• Languages: English
• ISSNs: 0040-6376

Abstract

Abstract: 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.