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

Takayuki Yoshida; Satoshi Konno; Ichizo Tsujino; Takahiro Sato; Hiroshi Ohira; Fengshi Chen; Hiroshi Date; Akihiro Ishizu; Hironori Haga; Mishie Tanino; Masaharu Nishimura

doi:10.2169/internalmedicine.53.1772

CASE REPORTS

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

Takayuki Yoshida, Satoshi Konno, Ichizo Tsujino, Takahiro Sato, Hiroshi Ohira, Fengshi Chen, Hiroshi Date, Akihiro Ishizu, Hironori Haga, Mishie Tanino, Masaharu Nishimura

Author information

Takayuki Yoshida
First Department of Medicine, Hokkaido University School of Medicine, Japan
Satoshi Konno
First Department of Medicine, Hokkaido University School of Medicine, Japan
Ichizo Tsujino
First Department of Medicine, Hokkaido University School of Medicine, Japan
Takahiro Sato
First Department of Medicine, Hokkaido University School of Medicine, Japan
Hiroshi Ohira
First Department of Medicine, Hokkaido University School of Medicine, Japan
Fengshi Chen
Department of Thoracic Surgery, Kyoto University, Japan
Hiroshi Date
Department of Thoracic Surgery, Kyoto University, Japan
Akihiro Ishizu
Faculty of Health Sciences, Hokkaido University, Japan
Hironori Haga
Department of Diagnostic Pathology, Kyoto University Hospital, Japan
Mishie Tanino
Department of Cancer Pathology, Hokkaido University Graduate School of Medicine, Japan
Masaharu Nishimura
First Department of Medicine, Hokkaido University School of Medicine, Japan

Keywords: pulmonary hypertension, pulmonary Langerhans cell histiocytosis, sildenafil

JOURNAL OPEN ACCESS

2014 Volume 53 Issue 17 Pages 1985-1990

DOI https://doi.org/10.2169/internalmedicine.53.1772

Details

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.

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