SESSION TITLE: Miscellaneous Global Case Reports
SESSION TYPE: Global Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Idiopathic pleuorparanchymal fibroelastosis is a very rare disease defined recently which is characterized pleural and subpleural parenchymal fibrosis predominantly on upper lobes. Marked apical pleural thickening associated with superior hilar retraction is present at chest X ray analysis and pleural thickening, fibrosis, architectural distortion, traction bronchiectasis and honeycomb lung.in high resolution computed tomography are radiological findings of this rare disease. We present a very rare case of idiopathic pleuraparanchymal fibroelastosis defined according to the radiological findings.
CASE PRESENTATION: We present a twenty two year-old male patient admitted with the complaint of progressive dyspnea since 9 months. He is a nonsmoker, he had any exposures to asbestosis, drugs or he had no environmental exposures. In his physical examination bibasillar crackles were determined. Chest X-ray showed bilateral reticular pattern and bilateral apically located pneumothorax. He had moderate hypoxemia on his arterial blood gases tests. In the thorax CT, bilateral apically located pneumothorax, interstitial fibrotic changes on upper lobes and tractional bronchiectasis were determined. Pulmonary function tests revealed severe restriction, and very low diffusing capacity. Six minute walking distance was 220 m with a severe desaturation. Immunological markers were negative for collagen tissue disorders. The radiological evaluation of this case suggested the diagnosis of “Idiopathic pleuorparanchymal fibroelastosis”. The only therapeutic option is lung transplantation, and we discussed this option with our patient, however he didn’t accept. He is on home oxygen therapy and pirfenidone was started, after a 3 month treatment period a- follow-up evaluation will be planned.
DISCUSSION: This rare form of fibrosis involving pleura and lung parenchyma had been suggested by the thorax radiologist which is characterized pleural and subpleural parenchymal fibrosis predominantly on upper lobes. We haven't perform an open lung biopsy since it could cause serious complications described in the literature (prolonged air leaks...).The differential diagnosis of IPPFE includes: asbestosis, connective tissue diseases, advanced fibrosing sarcoidosis, radiation or drug induced lung disease and chronic hypersensitivity pneumonitis. In this case any of this conditions was present. We describe the characteristic features of idiopathic pleural fibroelastosis radiologically.
CONCLUSIONS: This case presentation aimed to describe radiological and clinical features of this rare and progressive disease.
Reference #1: Piciucchi S et al. High resolution CT and histological findings in idiopathic pleuroparanchymal fibroelastosis features and differential diagnosis
DISCLOSURE: The following authors have nothing to disclose: Elif Sen, Ugur Gonullu, Cetin Atasoy, Hatice Taslak, Ozlem Ozdemir Kumbasar
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