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Pulmonary Langerhans cell histiocytosis (PLCH): a new UK register
Rebecca H Mason1, Noeleen M Foley2, Howard M Branley3
1Respiratory Department, Musgrove Park Hospital, Taunton, Somerset, UK.
Insights
Pulmonary Langerhans cell histiocytosis (PLCH) is a rare lung disease. Specialist centers may offer better outcomes for PLCH patients, with variations in treatment observed across UK hospitals.
Area of Science:
- Pulmonology
- Rare Diseases
- Oncology
Background:
- Pulmonary Langerhans cell histiocytosis (PLCH) is a rare interstitial lung disease with unknown causes.
- This study characterized a UK-wide cohort of PLCH patients to compare diagnostic and management approaches between specialist and non-specialist centers.
Discussion:
- A significant variation in clinical management and patient outcomes was observed, potentially linked to the level of clinical experience at different centers.
- Patients treated at specialist centers were more likely to receive cytotoxic drugs, and survival rates appeared higher in these centers.
Key Insights:
- The study identified 96% of patients as current or ex-smokers, highlighting the strong association between smoking and PLCH.
- A more even gender distribution was noted compared to previous documentation, with 53.7% of patients being female.
- Treatment modalities included smoking cessation (79%), corticosteroids (30.6%), cytotoxic therapy (26.9%), and lung transplantation (6%).
Outlook:
- Further research is needed to elucidate the unknown etiology of PLCH.
- Standardizing diagnostic and management protocols across all centers could improve patient outcomes.
- Investigating the specific factors contributing to better survival in specialist centers is crucial.
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.
