To describe the radiologic findings of the rare idiopathic interstitial pneumonias (IIPs) and how they correlate with their histologic patterns ; To provide some differential diagnosis of these diseases.
the revised American Thoracic Society/European Respiratory Society classification of idiopathic interstitial pneumonias distinguished two rare IIPs: Idiopathic lymphoid interstitial pneumonia (LIP) and idiopathic pleuroparenchymal fibroelastosis (PPFE) .
 As these conditions are still poorly recognized,
they pose a diagnostic challenge so,
these societies also highlighted the importance of a multidisciplinary approach to their correct diagnosis that encompasses clinical,...
Findings and procedure details
PLEUROPARENCHYMAL FIBROELASTOSIS Radiographic Findings Chest radiographs are nonspecific for the diagnosis of PPFE,
most commonly revealing apical reticulation , upper lobe volume loss and consequently upward hilar retraction ( Fig. 3 ). HRCT Findings HRCT characteristically shows bilateral pleural thickening and subpleural reticulation ,
with upper lung predominance and accompanying volume loss causing upward hilar retraction ( Fig. 4 - 9). Associated features include architectural...
There is considerable overlap between the rare IIPs’ radiologic and pathologic features. The definite diagnosis of these entities requires an agreement of a multidisciplinary team.
Contact Details: Dr.
Miguel Correia da Silva Resident in training.
Department of Radiology,
Centro Hospitalar de São João -Alameda Prof.
Portugal Email: firstname.lastname@example.org Department of Radiology,
Centro Hospitalar de São João: Thoracic Radiology Section: Dr.
Cunha Resident in training: Dr.
Carvalho Department of Pathology,
Centro Hospitalar de São João: Dr.
Travis WD, et al. An Official American Thoracic Society/European Respiratory Society Statement: Update of the International Multidisciplinary Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med, 2013; 188(6): 733-748 Reddy TL,
Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes.
Eur Respir J 2012; 40: 377–385 Bonifazi M,
Idiopathic Pleuroparenchymal Fibroelastosis.