sildenafil-citrate and Histiocytosis--Langerhans-Cell

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.

Topics: Biopsy; Diagnosis, Differential; Hemodynamics; Histiocytosis, Langerhans-Cell; Humans; Hypertension, Pulmonary; Lung; Lung Transplantation; Male; Phosphodiesterase 5 Inhibitors; Piperazines; Preoperative Care; Purines; Severity of Illness Index; Sildenafil Citrate; Sulfonamides; Tomography, X-Ray Computed; Young Adult

A 51-year-old woman (Case A) who had suffered from pulmonary Langerhans' cell granulomatosis for four years presented with progressive exertional dyspnea. A 39-year-old man (Case B) with pulmonary Langerhans' cell granulomatosis for eight years presented with right heart failure.. In Case A we found fixed airflow obstruction, hypoxemia, pulmonary fibrosis, bronchiectasis, severe precapillary pulmonary hypertension and a reduced right ventricular function, in Case B moderate airflow obstruction, hypoxemia, pulmonary fibrosis, emphysema and severe precapillary pulmonary hypertension.. Pulmonary hypertension due to pulmonary Langerhans' cell granulomatosis was assumed in both patients. After treatment with sildenafil, the woman has experienced long-term improvement for 2.5 years so far. An initially planned lung transplantation was therefore postponed. During inhaled iloprost the man also showed hemodynamic and clinical improvement and was stable for several years. Five years later recurrent right heart failure required an increase in iloprost dose; lung transplantation was then performed.. Although no approved drug is available for pulmonary hypertension in Langerhans' cell granulomatosis specific targeted therapies can lead to hemodynamic and long-term clinical improvement. Treatment could be helpful for selected patients with pulmonary hypertension in Langerhans' cell granulomatosis.

Topics: Adult; Drug Therapy, Combination; Female; Histiocytosis, Langerhans-Cell; Humans; Hypertension, Pulmonary; Iloprost; Male; Middle Aged; Piperazines; Purines; Sildenafil Citrate; Sulfones; Treatment Outcome; Vasodilator Agents

Topics: Antihypertensive Agents; Bosentan; Dyspnea; Hemodynamics; Histiocytosis, Langerhans-Cell; Humans; Hypertension, Pulmonary; Male; Middle Aged; Piperazines; Purines; Severity of Illness Index; Sildenafil Citrate; Sulfonamides; Sulfones