Effects of Iloprost and Sildenafil Citrate in A Patient with Pulmonary Hypertension Secondary to Histiocytosis X

Pulmonary hypertension secondary to pulmonary histiocytosis X is a progressive disease and has a poor prognosis. Despite conventional treatments (immunosuppresive agents, calcium channel blockers and continious O2 therapies) symptomatic improvement may not be seen. We found that single dose iloprost inhalation and oral sildenafil citrate treatment decreased pulmonary artery pressure in a patient with pulmonary hypertension secondary to histiocytosis X whose functional capacity was NYHA class IV. After one week of treatment period, functional capacity and six minutes walking test were improved with this combination. Combination of iloprost inhalation and oral sildenafil citrate might be an effective treatment options in pulmonary hypertension secondary to histiocytosis X.