Various Bronchiolar Lesions Accompanied by Idiopathic Pleuroparenchymal Fibroelastosis with a Usual Interstitial Pneumonia Pattern Demonstrating Acute Exacerbation

Abstract

A 63-year-old man presented with persistent cough and progressive dyspnea. Computed tomography showed irregular pleural thickening and fibrotic changes with volume loss in the upper lobes, and subtle reticulation in the lower lobes. Pleuroparenchymal fibroelastosis (PPFE) was diagnosed based on the findings of a surgical lung biopsy. Bronchiolar lesions, including proliferative bronchiolitis, constrictive bronchiolitis obliterans, and peribronchiolar metaplasia were evident on pathology. A usual interstitial pneumonia (UIP) pattern was also observed in the lower lobes. Three weeks after the biopsy, an acute exacerbation occurred. We herein describe a rare case of idiopathic PPFE with various bronchiolar lesions and a UIP pattern in which an acute exacerbation developed.

Acute Exacerbation of Pleuroparenchymal Fibroelastosis Secondary to Allogenic Hematopoietic Stem Cell Transplantation

“…We eventually suspected that the patient had AE based on the diagnostic criteria for AE-IPF ( 3 ), which are widely adopted for diagnosing AE in other ILDs including nonspecific interstitial pneumonia, chronic hypersensitivity pneumonitis, connective tissue disease associated ILDs, and unclassified interstitial pneumonia ( 4 - 7 ). To date, several individual cases with PPFE suspected to have AE have been reported ( 10 , 12 , 29 , 30 ). Although most of these episodes were AE that occurred in patients with iPPFE and a concomitant UIP pattern, Nei and colleagues reported AE in a patient with iPPFE without any evidence of other coexisting fibrotic ILDs ( 30 ).…”

An Autopsy Case of Idiopathic Pleuroparenchymal Fibroelastosis with Left Vocal Cord Paralysis and a Rapid Deterioration without an Acute Exacerbation

“…The organizing pneumonia was localized in the right lower lobe. There were no hyaline membranes, diffuse interstitial edema and organization, diffuse pneumocyte hyperplasia with squamous metaplasia, arterial thrombosis, or honeycombing suggesting acute exacerbation of interstitial pneumonia (6).…”

“…Patients with these diseases are characterized by 1) a slender stature with flat rib cage; 2) progressive bilateral fibrosis causing volume loss, mainly in the upper lung lobes; 3) predominant fibrosis of the subpleural parenchyma; 4) multiple bullae without honeycombing; 5) recurrent pneumothorax; 6) absence of extrathoracic lesions; 7) absence of identified acid-fast bacteria; 8) possible complication with aspergillus infection; and 9) slow progression, over approximately 10-20 years. Although the disease appears to progress gradually, the prognosis of IPPFE has varied widely among reported case studies ( 2 ), with some cases showing rapid deterioration ( 2 - 5 ); however, few patients die within a year of onset of the disease ( 4 - 6 ). In cases of IPPFE, progression of the disease itself is usually considered the cause of death, but the precise cause has been rarely discussed．…”

Pleuroparenchymal fibroelastosis

“…However, the risk of thoracic surgery and especially pleural complications and relapsing pneumothorax has become apparent in recent years. Acute exacerbation of fibrosis may also occur following surgical lung biopsy, although the risk might be related to the associated UIP pattern rather than to the PPFE component [57]. As a consequence, most centers experienced in PPFE now discourage to perform a video-assisted thoracoscopic lung biopsy, unless potential benefits and expected changes in management outweigh the risks.…”