Treatment of pulmonary LCH

Smoking cessation. Smoking cessation is mandatory because of the apparent causal effect of smoking in pulmonary LCH.[45] Most adult patients with LCH have gradual disease progression with continued smoking. The disease may regress or progress with the cessation of smoking.[46] A study of 27 patients with pulmonary LCH observed that 52% of patients improved after a mean follow-up of 14 months. Most patients improved with smoking cessation, and some patients improved with steroid treatment. Four patients (15%) had stable disease at a mean follow-up of 26 months, and nine patients (33%) demonstrated disease progression during the mean follow-up of 22 months.[30][Level of evidence C3]

Steroid therapy. It is not known whether steroid therapy is efficacious in the treatment of adult pulmonary LCH because reported case series did not control for smoking cessation.[45]

Chemotherapy. Some patients have been reported to respond to treatment with cladribine or cytarabine.[45,47]; [15][Level of evidence C2]

Lung transplant. Lung transplant may be necessary for adults with extensive pulmonary destruction from LCH.[22] One multicenter study reported a survival rate of 54% at 10 years posttransplant, with 20% of patients having recurrent LCH that did not impact survival. Longer follow-up of these patients is needed.[22] Another study confirmed a survival rate of approximately 50% at 10 years and improved hemodynamic changes associated with pulmonary arterial hypertension therapies, without oxygen worsening or pulmonary edema.[48]