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Background <p>Pleuroparenchymal fibroelastosis (PPFE) is a rare and distinct form of Interstitial lung disease predominantly affecting the upper lung zones. It is characterized by fibrotic thickening of the visceral pleura and adjacent subpleural parenchyma. While common complications include spontaneous pneumothorax and pneumomediastinum, vocal cord paralysis (VCP) or paresis has been increasingly recognized as a potential manifestation in recent reports.</p> Case presentation <p>We present a 49-year-old man presenting with progressive dyspnea, hoarseness, and left-sided vocal cord paralysis. Imaging studies revealed upper lobe-dominant fibrotic changes associated with significant pleural thickening consistent with PPFE. A comprehensive evaluation ruled out secondary causes of PPFE and other potential etiologies of VCP. Despite supportive management, the patient’s VCP persisted, likely due to architectural distortion of the lung affecting the recurrent laryngeal nerve pathway.</p> Conclusions <p>This case adds to the limited but growing body of literature on the association between PPFE and VCP. Understanding this rare complication is crucial for early recognition and appropriate management, as it highlights the diverse clinical manifestations of PPFE and its impact on patient outcomes.</p>

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A middle-aged man with dyspnea and hoarseness: an unusual case of vocal cord paralysis

  • 期刊论文

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  • 发表日期：

    2025年2月11日

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  • Seyedeh Nadia Tabatabaeifar,
  • Mahsa Ahmadpour,
  • Mohammad Javad Fallahi

摘要

Background

Pleuroparenchymal fibroelastosis (PPFE) is a rare and distinct form of Interstitial lung disease predominantly affecting the upper lung zones. It is characterized by fibrotic thickening of the visceral pleura and adjacent subpleural parenchyma. While common complications include spontaneous pneumothorax and pneumomediastinum, vocal cord paralysis (VCP) or paresis has been increasingly recognized as a potential manifestation in recent reports.

Case presentation

We present a 49-year-old man presenting with progressive dyspnea, hoarseness, and left-sided vocal cord paralysis. Imaging studies revealed upper lobe-dominant fibrotic changes associated with significant pleural thickening consistent with PPFE. A comprehensive evaluation ruled out secondary causes of PPFE and other potential etiologies of VCP. Despite supportive management, the patient’s VCP persisted, likely due to architectural distortion of the lung affecting the recurrent laryngeal nerve pathway.

Conclusions

This case adds to the limited but growing body of literature on the association between PPFE and VCP. Understanding this rare complication is crucial for early recognition and appropriate management, as it highlights the diverse clinical manifestations of PPFE and its impact on patient outcomes.