Long-term improvement during tadalafil therapy in a patient with pulmonary hypertension secondary to pulmonary Langerhans cell histiocytosis

• Published in: Respiratory medicine case reports 2016-01, Vol.18, p.54-57
• Main Authors: Nemoto, Kenji, Oh-ishi, Shuji, Inui, Toshihide, Nakazawa, Mariko, Hyodo, Kentaro, Nakajima, Masayuki, Kanazawa, Jun, Miura, Yukiko, Takaku, Takio, Minami, Yuko, Hayashihara, Kenji, Saito, Takefumi, Kawabata, Yoshinori
• Format: Article
• Language: English
• Subjects: Case Report; Internal Medicine; Phosphodiesterase-5 inhibitor; Pulmonary arterial hypertension; Pulmonary langerhans cell histiocytosis; Pulmonary/Respiratory; Tadalafil
• ISSN: 2213-0071; 2213-0071
• DOI: 10.1016/j.rmcr.2016.04.008

Abstract Pulmonary arterial hypertension (PAH) secondary to pulmonary Langerhans cell histiocytosis (PLCH) is known to be a relatively common complication and is associated with a poor prognosis. However, the optimal therapeutic approach for these cases remains to be established. A 57-year-old man visited our hospital because of a progressive dry cough. A thoracic computed tomography examination showed a combination of diffuse thick-walled cysts and reticulonodular shadows that were predominant in bilateral upper lobes of the lungs. He was diagnosed as having PLCH based on the results of video-assisted thoracoscopic lung biopsies. During a 3-year clinical course, his condition deteriorated despite smoking cessation. A systemic evaluation demonstrated precapillary PAH caused by PLCH (PAH-PLCH), and treatment with tadalafil, a phosphodiesterase-5 inhibitor, was started. During a 50-month period of treatment with tadalafil, improvements in his dyspnea, 6-min walking distance, and hemodynamics were maintained without either overt hypoxemia or pulmonary edema. We considered that tadalafil therapy may be a useful option in the treatment of patients with PAH-PLCH.