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

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• 作者：Kenji Nemoto^a ; ^{nemoken1124@yahoo.co.jp" class="auth_mail" title="E-mail the corresponding author} ; Shuji Oh-ishi^a ; Toshihide Inui^a ; Mariko Nakazawa^a ; Kentaro Hyodo^a ; Masayuki Nakajima^a ; Jun Kanazawa^a ; Yukiko Miura^a ; Takio Takaku^a ; Yuko Minami^b ; Kenji Hayashihara^a ; Takefumi Saito^a ; Yoshinori Kawabata^c
• 关键词：Pulmonary arterial hypertension ; Pulmonary langerhans cell histiocytosis ; Phosphodiesterase-5 inhibitor ; Tadalafil
• 刊名：Respiratory Medicine Case Reports
• 出版年：2016
• 出版时间：2016
• 年：2016
• 卷：18
• 期：Complete
• 页码：54-57
• 全文大小：1329 K

文摘

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.