PT  - JOURNAL ARTICLE
AU  - Mhairi Barclay
AU  - Rebecca Devaney
AU  - Jayesh. M. Bhatt
TI  - Paediatric pulmonary Langerhans cell histiocytosis
AID  - 10.1183/20734735.0003-2020
DP  - 2020 Jun 01
TA  - Breathe
PG  - 200003
VI  - 16
IP  - 2
4099  - http://breathe.ersjournals.com/content/16/2/200003.short
4100  - http://breathe.ersjournals.com/content/16/2/200003.full
SO  - Breathe2020 Jun 01; 16
AB  - Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a “risk organ” in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed.Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.Educational aimsTo give an overview of paediatric pulmonary LCH.To discuss the differential diagnosis of paediatric cystic lung disease.Paediatric pulmonary Langerhans cell histiocytosis is a rare diffuse lung disease. Involvement of the lungs is not associated with a worse prognosis than the involvement of other organs but management of respiratory complications are challenging. http://bit.ly/38mJHFq