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Volume: 6 Issue: 4 November 2008 - Supplement - 1



Idiopathic pleuroparenchymal fibroelastosis is a rare entity. It consists of fibrotic thickening of the pleura and subpleural parenchyma. The report of such phenomenon after lung transplantation has not been made. The patient was a 52 years old woman. She receives single lung transplantation on May 2007 because of end stage pulmonary fibrosis. One month after successful lung transplantation, she began to develop pleural thickening in the transplanted lung. The thickening progressed gradually and made significant restriction for the transplanted lung. She started having severe progressive dyspnea 13 months after lung transplantation. In plain chest radiogram and HRCT-Scan, severe pleural thickening at the base of the transplanted lung is obvious. Fibrotic band are also extended into the lung parenchyma. The course of the disease was progressive with no response to high-dose corticosteroid therapy. Previous reports of similar condition are scant and do not contain lung-transplanted patients. The radiographic features in plain chest radiogram and HRCT are similar with the exception that previous reports showed such phenomenon in upper lobes while the findings in our patient was more prominent at lower lobe. We did not observe any improvement in patient and disease condition using corticosteroid therapy. While lung transplantation therapy was reported as a treatment in some previous cases, we cannot justify such treatment in our patient regarding the fact that the illness occurred after and within the transplanted lung. Treatment of such illness remained a challenge and requires further investigation.

Volume : 6
Issue : 4
Pages : 229

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Transplant Research Center, Massih Daneshvari Hospital, Darabad-Niavaran, Tehran, Iran