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Pulmonary Langerhans cell histiocytosis (PLCH): a new UK register
  1. Rebecca H Mason1,
  2. Noeleen M Foley2,
  3. Howard M Branley3,
  4. Huzaifa I Adamali4,
  5. Martin Hetzel5,
  6. Toby M Maher6,
  7. Jay Suntharalingam2
  1. 1Respiratory Department, Musgrove Park Hospital, Taunton, Somerset, UK
  2. 2Respiratory Department, Royal United Hospital, Bath, UK
  3. 3Respiratory Department, Ealing Hospital NHS Trust, London, Middlesex, UK
  4. 4Bristol Interstitial Lung Disease (BILD) Service, North Bristol Lung Centre, Southmead General Hospital, Bristol, UK
  5. 5Respiratory Department, Bristol Royal Infirmary, Bristol, UK
  6. 6Interstitial Lung Disease Unit, Royal Brompton, London, UK
  1. Correspondence to Dr Jay Suntharalingam, Respiratory Department, Royal United Hospital, Bath BA1 3NG, UK; Jay.suntharalingam{at}nhs.net

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.

  • Rare lung diseases
  • Interstitial Fibrosis

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