Idiopathic Pleuroparenchymal Fibroelastosis

Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare form of idiopathic interstitial pneumonia. The disease is characterized by fibrosis of the pleura and subpleural lung parenchyma predominantly affecting the upper lobes. Various triggers have been proposed as inciting factors in the development of the disease. Diagnosis is made clinically in conjunction with radiographic findings and histopathology when available. There are no known effective treatment options and several cases of lung transplantation have been reported. We report a case of an 86-year-old female who presented to the emergency department with worsening dyspnea and hypoxia. She had a history of unexplained pneumomediastinum and a 20 - 25 pounds unintentional weight loss over 10 months. Computed tomography (CT) of the chest without contrast revealed radiographic evidence of IPPFE. Despite symptomatic management with antibiotics, diuretics, and steroids, her condition continued to deteriorate. Unfortunately, our patient was not a candidate for a lung transplant. She was transitioned to hospice care and succumbed to her disease. IPPFE is a rare disease with an unknown prevalence. It has a median survival rate of 2 years. Usually, there is an overlap with interstitial lung diseases, making it challenging to diagnose. There are only a few cases reported in the literature, and there are currently no guidelines available on the appropriate management of this debilitating disease. We recommend more cases be reported, and further research is done to establish better criteria for diagnosis and management.

Keywords: Dyspnea; Hypoxia; Idiopathic pleuroparenchymal fibroelastosis; Interstitial lung disease; Interstitial pneumonia; Platythorax; Pneumomediastinum; Pneumothorax.

Figure 1

Chest X-ray posterior-anterior (a) and lateral (b) views showing cardiomegaly, pulmonary vascular congestions, and small bilateral pleural effusions (red arrows).

Figure 2

Coronal (a) and axial (b) computed tomography (CT) scan of the chest demonstrating bilateral apical pleural thickening (green arrows).

Figure 3

Computed tomography (CT) scan of the chest showing upper lobe predominant interstitial lung disease with thickened pleura (blue arrows), as well as traction bronchiectasis, basilar reticulations, and honeycombing (red arrows). Right lung opacities (green arrows) are observed in the location of the prior radiation treatment.