title: Long-term improvement during tadalafil therapy in a patient with pulmonary hypertension secondary to pulmonary Langerhans cell histiocytosis.
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.
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Nemoto
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Shuji
authors.initials: S
authors.lastname: Oh-Ishi
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Toshihide
authors.initials: T
authors.lastname: Inui
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Mariko
authors.initials: M
authors.lastname: Nakazawa
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Kentaro
authors.initials: K
authors.lastname: Hyodo
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Nakajima
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Kanazawa
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Miura
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Takio
authors.initials: T
authors.lastname: Takaku
authors.affiliation: Department of Pathology, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Minami
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Hayashihara
authors.affiliation: Department of Respiratory Medicine, National Hospital Organization, Ibarakihigashi National Hospital, Ibaraki, Japan.
authors.forename: Takefumi
authors.initials: T
authors.lastname: Saito
authors.affiliation: Division of Diagnostic Pathology, Saitama Prefectural Cardiovascular and Respiratory Centre, Saitama, Japan.
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Kawabata
keywords: Phosphodiesterase-5 inhibitor; Pulmonary arterial hypertension; Pulmonary langerhans cell histiocytosis; Tadalafil