Idiopathic pleuroparenchymal fibroelastosis presenting in recurrent pneumothorax: a case report

Abstract

Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare, recently classified entity that consists of pleural and subjacent parenchymal fibrosis predominantly in the upper lungs. In an official American Thoracic Society/European Respiratory Society statement in 2013, this disease is introduced as a group of rare idiopathic interstitial pneumonias. We describe a case of a 76-year-old woman with cough and recurrent pneumothorax. She was admitted to our hospital with severe cough at first. High resolution computed tomography (HRCT) disclosed multifocal subpleural consolidations with reticular opacities in both lungs, primarily in the upper lobes, suggesting interstitial pneumonia. Rheumatoid lung was diagnosed initially through an elevated rheumatoid factor, HRCT and surgical biopsy at the right lower lobe. However, one month later, pneumothorax recurred. Surgical biopsy was performed at the right upper lobe at this time. The specimens revealed typical subpleural fibroelastosis. We report this as a first case of idiopathic PPFE in Korea after reviewing the symptoms, imaging and pathologic findings.

Keywords: Fibrosis; Idiopathic Interstitial Pneumonias; Pneumothorax.

Figure 1

Chest radiograph shows multifocal consolidations and reticulonodular infiltrations in peripheral portion of both lungs. These lesions show upper lobe predominancy. Small pneumothorax is noted in right lung.

Figure 2

(A) High resolution computed tomography (HRCT) shows multifocal subpleural consolidations with reticular opacities in both lungs and small pneumothorax in right lung. Architectural distortions and traction bronchiectasis are combined in both lungs. (B) Coronal HRCT shows that these lesions predominantly affect the upper lobes.

Figure 3

Surgical lung biopsy specimen (H&E stain, ×40) at low power demonstrated dense fibrosis in the subpleura, lung parenchyme, and interstitium with temporal heterogeneity.

Figure 4

A chest radiagraph shows an increased amount of pneumothorax in the right lung compared with Figure 1.

Figure 5

Surgical lung biopsy specimen (Elastic Van Gieson stain) at low power (A, ×100) shows pleural thickening and subpleural fibrosis. High power (B, ×200) demonstrated dense masses of elastic fibers beneath the thickend pleura.