Related Experiment Video
Updated: May 19, 2026
07:51
Refined Murine Model of Idiopathic Pulmonary Fibrosis
Published on: June 17, 2025
Acute Exacerbation of Unilateral Upper-lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal
Keigo Matsuura1, Akimasa Sekine1, Eri Hagiwara1
1Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Japan.
Internal Medicine (Tokyo, Japan)
|May 17, 2026
Summary
This study highlights a rare case of acute exacerbation in unilateral upper-lung field pulmonary fibrosis (upper-PF), a condition typically associated with pleuroparenchymal fibroelastosis. Physicians should consider this possibility even in unilateral cases.
Area of Science:
- Pulmonology
- Radiology
- Pathology
Background:
- Pleuroparenchymal fibroelastosis (PPF) is a rare form of idiopathic pulmonary fibrosis.
- Upper-lung field pulmonary fibrosis (upper-PF) can present unilaterally after thoracic surgery.
- Acute exacerbations of idiopathic pulmonary fibrosis are well-documented but rare in unilateral PPF.
Purpose of the Study:
- To report a rare case of acute exacerbation in a patient with unilateral upper-PF.
- To emphasize the importance of recognizing acute exacerbations in this specific patient population.
- To discuss the diagnostic and therapeutic considerations for such cases.
Main Methods:
- Case report of a 58-year-old male patient.
- Postoperative follow-up including imaging (Chest CT).
- Diagnosis of acute exacerbation of unilateral upper-PF after excluding other causes of respiratory failure.
Main Results:
- Development of unilateral upper-PF post-lobectomy for lung cancer.
- Seventeen years later, patient presented with acute dyspnea and bilateral ground-glass opacities.
- Diagnosis of acute exacerbation of unilateral upper-PF confirmed and successfully treated with anti-inflammatory therapy.
Conclusions:
- Acute exacerbation can occur in patients with unilateral upper-PF, albeit rarely.
- Chest physicians must consider acute exacerbations in patients with unilateral upper-PF.
- Prompt diagnosis and anti-inflammatory treatment can be effective.
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