Diagnosis of pleuroparenchymal fibroelastosis: A review

Abstract

Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease with unprecedented features characterized by fibroelastotic changes in the subpleural lung parenchyma affecting the upper lobes. PPFE is usually idiopathic, but it can be caused by infection, autoimmunity, bone marrow or lung transplantation, or a genetic predisposition. Histopathologic examination of lung biopsy samples reveals homogenous subpleural fibrosis and abundant elastic fibers, allowing for a definitive diagnosis. As PPFE mimics many interstitial lung diseases, clinicians face significant difficulties in making a definitive final diagnosis. Since most disease-related comorbid conditions manifest at an advanced stage, invasive tissue sampling for histopathologic evaluation is consistently impossible. Such a patient presentation highlights the importance of an analysis based solely on clinical findings, which would provide a definitive diagnosis without the need for a biopsy. Because of its exceptional and inconceivable presentation, PPFE creates a diagnostic dilemma. In light of our two cases and the literature data, we present a diagnostic assessment score assay that relies solely on clinical manifestations without histopathological tissue verification to shed light on the diagnosis of PPFE. This review focuses on PPFE identification through the use of a diagnostic assessment analysis to improve early disease recognition without the use of invasive diagnostic interventions to obtain biopsy samples for histopathologic evaluation. This analytic approach, while not diagnostic in and of itself, may provide a useful pathway for differential diagnosis and may preclude redundant initiatives.

Clinical Diagnosis of Pleuroparenchymal Fibroelastosis

“…Since this situation necessitates making the diagnosis according to clinical manifestations in the vast majority of patients, the diagnostic evaluation score we have introduced provides a great support to clinicians both for the definitive diagnosis of the disease and the differential diagnosis with other ILDs. We have reached an accurate defingtive diagnosis in two PPFE patients by using the diagnostic assessment score analysis (Tables 1 and 2) without the slightest reqiurement for tissue biopsy [35,36]. This analytical approach, besides its usefulness in terms of an accurate and definitive diagnosis, provides an effective approach to clinicians by creating a diagnostic pathway for the differential diagnosis with other interstitila lung diseases and for the evaluation of suspicious pleuroparenchymal fibroelastosis cases to preclude the redundant diagnostic interventions.…”

“…Posterior tracheal border and spine convergence along with a notable deep suprasternal notch appearence may develop because of reduced upper thoracic volume and progressive weight loss that emerge as the other crucial diagnostic HRCT findings tissue biopsy evaluation [35] while a recent version of this algorithm comprising more objective diagnostic criteria is under publication. A new diagnostic algorithm, which includes more precise and objective diagnostic criteria for a definitive and accurate diagnosis of PPFE is still under publication [36].…”

“…Thorax CT is emerges as the most useful and definitive imaging modality for PPFE and is the hallmark of clinical diagnosis. deepening [36].…”

“…Figure 3. Thorax CT findings of the PPFE patient showing pleural thickening, pneumothorax, subpleural fibrosis, and suprasternal notchdeepening[36].…”

“…Figure 2. Thorax CT manifestations of the PPFE revealing pleuralthickening, subpleural parenchymal fibrosis, platyhorax, and suprasternal notch deepening[36].…”