European Journal of Biomedical and Pharmaceutical Sciences

A VERY RARE CASE OF PLEURO-PARENCHYMAL FIBROELASTOSIS (PPFE)

Dr. Ritamvara Oli***, Prof. J. K. Mishra**, Prof. G. N. Srivastava*, Dr. Deepak Shah#, Dr. Saurabh Mishra***, Dr. Deepanjali Sharma***

ABSTRACT

Pleuro-parenchymal fibroelastosis (PPFE) is a rare ILD added to a subgroup of Idiopathic interstitial pneumonia. It is a rare disease usually involving the upper lobe. It has been found to affect the visceral pleura causing fibrosis and adjacent lung parenchyma causing fibroelastosis of the lining of the lung. Till date only 120 cases have been reported in world literature. Etiology remains unknown but has also been associated with bone marrow transplant patients, infection and autoimmunity. The median age of presentation is 53 years. Patient presents with a long history of persistent shortness of breath on exertion, dry cough. Around 1/3 patient presents with Pneumothorax. Patient is usually thin built. PFT reveals restrictive pattern. CT scan shows Upper and middle lung zone pleural and subpleural reticular parenchymal fibrosis with demarcation between affected and unaffected zone. Diagnosis is made clinically, radiologically and histologically.

Keywords: Pleuro-parenchymal fibroelastosis (PPFE), Idiopathic interstitial pneumonia, Interstitial lung disease (ILD).