A rare cause of pulmonary hypertension: pulmonary Langerhans cell granulomatosis

Pulmonary Langerhans cell granulomatosis (PLCG) is a smoking-related interstitial lung disease characterized by hyperinflation and/or obstructive pulmonary disease. Pulmonary hypertension is a late complication of advanced pulmonary disease and often portends a poor prognosis. A 24-year-old male patient who had been receiving diltiazem and corticosteroid treatment with the diagnosis of PLCG for four years was examined by the cardiology clinic due to dyspnea and deterioration in his functional capacity. He was in New York Heart Association class III. The electrocardiogram showed sinus rhythm and right bundle branch block. Systolic pulmonary artery pressure (SPAP) was estimated as 80 mmHg with continuous-wave Doppler echocardiography. During right heart catheterization, SPAP was 70 mmHg and the mean pulmonary artery pressure (MPAP) was 44 mmHg. Vascular pulmonary reactivity test with adenosine did not result in a significant change in MPAP. Based on these findings, medical treatment was scheduled for the patient.