Table 1

Therapies and medical interventions for PLCH

Clinical context
Smoking cessationMandatory for all cigarette smokers. Avoidance of all second-hand smoke exposure highly recommended
  InhaledPatients with concomitant COPD or airflow limitation
  OralLimited role in most clinical contexts: may have role for treatment of some multisystem forms of disease
OxygenPatients with hypoxemia (O2sat<89%) at rest or with activity, particularly in individuals with pulmonary hypertension
Pulmonary hypertension therapiesNo clear consensus regarding use. Systemic vasodilator therapy should only be considered in selected patients with pulmonary hypertension following right heart catheterisation and vasodilator challenge. Inhaled prostacyclin may be more beneficial in this context, but data regarding safety and efficacy are lacking
Cladribine (2-CDA)Consider for patients with abnormal lung function, if disease progression occurs despite smoking cessation, and in patients with multsystem disease
CytarabineReported efficacy in anecdotal reports and series of patients with multi-system Langerhans cell histiocytosis and pulmonary involvement
VinblastineLimited evidence for efficacy
Lung transplantationConsideration in any patients with advanced and/or progressive disease in spite of smoking cessation or medical therapy