TY - JOUR T1 - Pulmonary Langerhans cell histiocytosis (PLCH): a new UK register JF - Thorax JO - Thorax SP - 766 LP - 767 DO - 10.1136/thoraxjnl-2013-204313 VL - 69 IS - 8 AU - Rebecca H Mason AU - Noeleen M Foley AU - Howard M Branley AU - Huzaifa I Adamali AU - Martin Hetzel AU - Toby M Maher AU - Jay Suntharalingam Y1 - 2014/08/01 UR - http://thorax.bmj.com/content/69/8/766.abstract N2 - 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. ER -