Airway-centered Fibroelastosis Accompanied by Subpleural Lesions of Unknown Cause in a Young Man Who Later Developed Pulmonary Hypertension

Shojiro Minomo; Toru Arai; Kazunobu Tachibana; Hideo Matsui; Takahiko Kasai; Masanori Akira; Yoshikazu Inoue

doi:10.2169/internalmedicine.3256-19

CASE REPORTS

Airway-centered Fibroelastosis Accompanied by Subpleural Lesions of Unknown Cause in a Young Man Who Later Developed Pulmonary Hypertension

Shojiro Minomo, Toru Arai, Kazunobu Tachibana, Hideo Matsui, Takahiko Kasai, Masanori Akira, Yoshikazu Inoue

Author information

Shojiro Minomo
Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Toru Arai
Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Kazunobu Tachibana
Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Hideo Matsui
Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Takahiko Kasai
Department of Pathology, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Masanori Akira
Department of Radiology, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Yoshikazu Inoue
Department of Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan
Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan

Keywords: airway-centered fibroelastosis, bronchial asthma, bronchial abnormality, pulmonary hypertension, subpleural lesions, upper lobes

JOURNAL OPEN ACCESS

2020 Volume 59 Issue 5 Pages 695-700

DOI https://doi.org/10.2169/internalmedicine.3256-19

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Abstract

A 26-year-old man with a history of bronchial asthma was found to have high-density shadows along the bronchovascular bundle and in the subpleural area on computed tomography of the chest. Surgical lung biopsy specimens from the right S⁵ showed fibroelastosis in the subpleural and central airway area with alveolar destruction. He was diagnosed with airway-centered fibroelastosis of unknown cause after multidisciplinary discussions. The patient developed pulmonary hypertension and died 6 years later. The patient was younger in comparison to patients in earlier reports and had more obvious subpleural fibroelastic lesions in the upper lobes than in previously described cases.

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