Therapies and medical interventions for PLCH
| Clinical context | |
| Smoking cessation | Mandatory for all cigarette smokers. Avoidance of all second-hand smoke exposure highly recommended |
| Corticosteroids | |
| Inhaled | Patients with concomitant COPD or airflow limitation |
| Oral | Limited role in most clinical contexts: may have role for treatment of some multisystem forms of disease |
| Oxygen | Patients with hypoxemia (O2sat<89%) at rest or with activity, particularly in individuals with pulmonary hypertension |
| Pulmonary hypertension therapies | No 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 |
| Cytarabine | Reported efficacy in anecdotal reports and series of patients with multi-system Langerhans cell histiocytosis and pulmonary involvement |
| Vinblastine | Limited evidence for efficacy |
| Lung transplantation | Consideration in any patients with advanced and/or progressive disease in spite of smoking cessation or medical therapy |