Severe Pulmonary Hypertension in Adult Pulmonary Langerhans Cell Histiocytosis: The Effect of Sildenafil as a Bridge to Lung Transplantation

Abstract

Severe pulmonary hypertension (PH) often develops in patients with pulmonary Langerhans cell histiocytosis (PLCH). Supplemental oxygen treatment is often used, whereas pulmonary arterial hypertension-specific vasodilators are generally considered hazardous because of the possible development of pulmonary edema and deterioration of hypoxia. In the present report, we herein describe a PLCH patient with severe PH in whom sildenafil, a phosphodiesterase 5 (PDE5) inhibitor, substantially improved the pulmonary hemodynamics before lung transplantation. An immunohistochemical study of the resected lung revealed positive staining for PDE5 on the diseased pulmonary arteries. These observations suggest that sildenafil can be a promising therapeutic option for PH in patients with PLCH.

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

“…Conversely, some papers reported good clinical and hemodynamic outcomes with PAH-specific drugs in these patients (4)(5)(6)8). In addition, these drugs can be used as a bridging therapy to lung transplantation, although in the absence of prospective or head-to-head comparisons of PAH-specific therapies in PLCH, endothelin receptor antagonists and phosphodiesterase-5 inhibitors seem to be effective (8,9). In our patient, we observed sustained improvement in hemodynamic and clinical parameters with PAH treatments, and this benefit was obtained without any deterioration in oxygen saturation.…”

“…epoprostenol was tried in a very small number of patients and resulted in pulmonary edema (7). Conversely, some papers reported good clinical and hemodynamic outcomes with PAH-specific drugs in these patients (4)(5)(6)8). In addition, these drugs can be used as a bridging therapy to lung transplantation, although in the absence of prospective or head-to-head comparisons of PAH-specific therapies in PLCH, endothelin receptor antagonists and phosphodiesterase-5 inhibitors seem to be effective (8,9).…”

Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation

“…The absence of vascular changes in areas uninvolved in Langerhans cell granulomas makes this case distinct. Yoshida et al reported a PLCH-PH patient in whom vascular changes were observed in both involved and uninvolved areas (10). In their case, smoking cessation was not effective, but sildenafil substantially improved the pulmonary hemodynamics.…”