Pulmonary Langerhans cell histiocytosis (PLCH): a new UK register.
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.
Respiratory Department, Musgrove Park Hospital, Taunton, Somerset, UK.
Rebecca H
RH
Mason
Respiratory Department, Royal United Hospital, Bath, UK.
Noeleen M
NM
Foley
Respiratory Department, Ealing Hospital NHS Trust, London, Middlesex, UK.
Howard M
HM
Branley
Bristol Interstitial Lung Disease (BILD) Service, North Bristol Lung Centre, Southmead General Hospital, Bristol, UK.
Huzaifa I
HI
Adamali
Respiratory Department, Bristol Royal Infirmary, Bristol, UK.
Martin
M
Hetzel
Interstitial Lung Disease Unit, Royal Brompton, London, UK.
Toby M
TM
Maher
Respiratory Department, Royal United Hospital, Bath, UK.
Jay
J
Suntharalingam
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000453:epidemiology / Q000628:therapy*; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D012042:Registries; D012307:Risk Factors; D016540:Smoking Cessation; D015996:Survival Rate; D006113:United Kingdom / Q000453:epidemiology
D000305:Adrenal Cortex Hormones
Interstitial Fibrosis; Rare lung diseases