Sustained response to targeted therapies in a patient with pulmonary hypertension owing to Langerhans cell histiocytosis

Abstract

Pulmonary hypertension (PH) in association with Pulmonary Langerhans Cell Histiocytosis (PLCH) is an uncommon pulmonary vascular disease classified in group 5 PH according to currently available guidelines for the diagnosis and treatment of PH. Oxygen support, diuretic treatment and lung transplantation are standard therapies for this disease. Generally, pulmonary arterial hypertension (PAH) targeted therapies are not considered to be beneficial in this subgroup of PH.