Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Pleuroparenchymal fibroelastosis
- Radiologic Findings
- Chest PA shows bilateral apical pleural thickening and subpleural opacities.
Chest CT images show bilateral subpleural irregular opacities, pleural thickening and traction bronchiolectasis in both upper lobes. In the lower lobes there was no pleural irregularity, but there was a subtle traction bronchiectasis.
VATS biopsy of the right upper lobe demonstrated fibroelstosis involving the pleura and subpleural lung parenchyma consistent with pleuroparenchymal fibroelastosis.
- Brief Review
- Pleuroparenchymal fibroelastosis (PPFE) include the category of rare idiopathic interstitial pneumonia with idiopathic lymphoid interstitial pneumonia. PPFE is a rare condition that consists of fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes. HRCT shows dense subpleural consolidation with traction bronchiectasis, architectural distortion, and upper lobe volume loss. Pleural fibrosis is associated with subjacent intra-alveolar lung parenchymal fibrosis and alveolar septal elastosis. Most common presentation is in adults with a median age of 57 years with no sex predilection. Approximately half of patients have history of recurrent infections. Pneumothorax may complicate the course of the disease and even recur. A minority may have familial interstitial lung disease and nonspecific autoantibodies. Histological biopsies sometime show mild changes of PPFE with UIP and NSIP. Disease process is progressive in 60% of patients and may be fatal 40%.
Special Note from Archana Laroia, Associate Professor of Iowa University Hospital, English copyeditor of weekly chest cases- I think the authors may want to consider including that bone marrow or stem cell transplantation and lung transplantation are now known to cause PPFE, as a lung manifestation of chronic graft versus host disease (GVHD) and chronic rejection of transplanted lungs.
- References
- Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, 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;188:733-748
- Keywords
- Lung, Idiopathic interstitial pneumonia, IIP,