title: Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation.
abstract: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare, smoking-related, interstitial lung disease, and pulmonary hypertension (PH) is associated with mortality. We herein report a case of PLCH complicated by severe PH and respiratory impairment. After developing PH, the patient displayed a cystic pattern on chest high-resolution computed tomography (HRCT). This, in turn, corresponded with the scarring stage of PLCH. However, the patient's PH and respiratory impairment improve dramatically following smoking cessation. PLCH patients with a cystic pattern on chest HRCT may still be able to improve their PH and respiratory impairment when they are able to quit smoking.
authors.affiliation: Division of Respiratory Medicine, Department of Internal Medicine, National Hospital Organization, Kokura Medical Center, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Kinoshita
authors.forename: Kentaro
authors.initials: K
authors.lastname: Watanabe
authors.forename: Atsuhiko
authors.initials: A
authors.lastname: Sakamoto
authors.forename: Kouko
authors.initials: K
authors.lastname: Hidaka
mesh_terms: D004232:Diuretics / Q000627:therapeutic use; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy; D008875:Middle Aged; D012131:Respiratory Insufficiency / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy; D012907:Smoking / Q000009:adverse effects*; D016540:Smoking Cessation; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D004232:Diuretics; D014665:Vasodilator Agents