CLINICAL IMAGE
A 75-year-old female patient was referred to our hospital by an otolaryngologist with a history of voice hoarseness resulting from left-sided vocal cord paralysis (VCP) and exertional dyspnea. Chest X-ray and chest computed tomography (CT) (Fig. 1) revealed bilateral upper-lobe dominant pleural thickening with subpleural fibrosis, volume loss, and upward tracheobronchial tree retraction; which are radiologic features consistent with pleuroparenchymal fibroelastosis (PPFE) [1]. She also reported symptoms such as breathlessness, weight loss (lost 10 kg over a year), and plathythorax, i.e. anteroposterior chest flattening; which were suggestive of PPFE. A reduction in forced vital capacity (51% predicted) with a restricted pattern was observed in the pulmonary function tests. Blood tests revealed the absence of autoimmune antibodies and nonelevated serum levels of Krebs von den Lungen-6. No other underlying causes of VCP, such as thyroid or lung cancer, mediastinal lymphadenopathy, or aortic aneurysms, were identified. The patient was diagnosed with idiopathic PPFE accompanying left VCP. PPFE is a rare lung disease characterized by predominant upper-lobe fibroelastosis and pleural thickening, with frequent complications of pneumothorax and pneumomediastinum [1]. Six cases of PPFE with VCP, including the present case, with symptoms of hoarseness [2–6] and occasionally aspiration pneumonia [3, 6] have been reported. VCP occurred predominantly on the left side in five out of the six cases, probably due to a longer intrathoracic path of the left recurrent laryngeal nerve (RLN) [2, 3, 5, 6]. Several mechanisms that have been proposed for the underlying VCP in PPFE are: RLN stretching or retraction due to its chest wall adhesion resulting from fibrosis [3], tracheobronchial tree distortion leading to RLN traction or compression [5, 6], and left RLN compression in the aortopulmonary window where the aortic arch and left pulmonary artery are in close contact (Fig. 1b) [5, 6]. PPFE should be considered as a differential diagnosis for VCP.
Figure 1.
Chest computed tomography images displaying pleural thickening with pulmonary fibrosis, volume loss, and traction bronchiectasis in the bilateral upper lobes (a, transverse view of lung window image; b, coronal view of mediastinal window image). Note that the aortic arch and left pulmonary artery are in close contact (arrows).
Supplementary Material
ACKNOWLEDGEMENTS
The authors declare no relevant acknowledgment.
Contributor Information
Reimi Mizushima, Department of Respiratory Medicine, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan; Department of Respiratory Medicine, Tokyo Medical University Hospital, Tokyo, Japan.
Rena Tamenaga, Department of Respiratory Medicine, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan; Department of Respiratory Medicine, Tokyo Medical University Hospital, Tokyo, Japan.
Taro Kufukihara, Department of Respiratory Medicine, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan; Department of Respiratory Medicine, Tokyo Medical University Hospital, Tokyo, Japan.
Yukihisa Takeda, Department of Respiratory Medicine, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan.
Yusuke Watanabe, Department of Respiratory Medicine, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan; Department of Infection Prevention and Control, Tokyo Medical University Hospital, Tokyo, Japan.
Hiroyuki Nakamura, Department of Respiratory Medicine, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan.
Kazutetsu Aoshiba, Department of Respiratory Medicine, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan.
CONFLICT OF INTEREST STATEMENT
There are no conflicts of interest.
FUNDING
No funding sources were used.
DATA AVAILABILITY STATEMENT
The data supporting the findings of this study are available from the corresponding author upon request; they are not publicly available due to privacy or ethical restrictions.
ETHICAL APPROVAL
This case report meets the standards of the Tokyo Medical University ethical committee. All personal identifiers were removed from the manuscript.
CONSENT
Written consent from the patient was obtained for the submission and publication of the case details and images.
GUARANTOR
The nominated guarantor is K.A.
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Data Availability Statement
The data supporting the findings of this study are available from the corresponding author upon request; they are not publicly available due to privacy or ethical restrictions.