History : 38-year-old male with progredient dyspnea, productive cough, frequent airway infections and restrictive ventilatory defect. BAL and Biopsy were.

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Presentation transcript:

History : 38-year-old male with progredient dyspnea, productive cough, frequent airway infections and restrictive ventilatory defect. BAL and Biopsy were negative for sarcoid. Patient underwent lung transplantation in Case of the Month 11 May 2016

Case of the Month 11 An initial chest radiograph was performed....

Case of the Month 11...followed by CTs 10/2008 5/2012 6/2010

Case of the Month 11...followed by CTs 10/2008 5/2012 6/2010

Case of the Month 11...followed by CTs 10/2008 5/2012 6/2010

Case of the Month 11 What is your diagnosis ?

 Marked bilateral apical parenchymal infiltrates and pleural thickening Case of the Month 11

 Marked bilateral apical pleural thickening  Reticular abnormalities, including  Thickening of interlobular septa  Architectural distortion, possibly leading to  Traction bronchiectasis  Almost no changes over the years, but a slight shrinking of the lung

Case of the Month 11 The patient had several biopsies of the upper lobe

Case of the Month 11  Histology of the lung parenchyma showed:  Abundance of short, curled and randomly oriented elastic fibers  Resulting in elastic fibrosis of the visceral pleura

Case of the Month 11 Diagnosis What is your diagnosis ?

Case of the Month 11 Diagnosis Pleuroparenchymal fibroelastosis Case provided by Dr Cejka and Professor Frauenfelder, Zurich Switzerland

Case of the Month 11 Discussion  Etiology and pathogenesis of pleuroparenchymal fibroelastosis is unknown  Average age at diagnosis is 50 years  Pleuroparenchymal fibroelastosis is s characterised by marked pleural and sub pleural fibrosis, typically in the upper lobes  Patients present with non-productive cough, dyspnea in a worsening course, frequent airway infections  It is a very rare disorder, however it is very likely both under recognized and misdiagnosed

Case of the Month 11 Discussion  Histopathological findings as marked thickening of the visceral pleura and prominent subpleural fibrosis with sparing of the parenchyma distant from the pleura are characteristic

Case of the Month 11 Discussion  CT is the imaging method of choice  Because of the rarity of the fibrosis, its clinical characteristics have not been fully elucidated  Imaging is impaired with clinic and shows almost equal findings for many years, but a slight shrinking of the lung  For definitive diagnosis thoracoscopic or open- lung biopsy is needed as pleuroparenchymal histopathology is characteristic

Case of the Month 11 Discussion CHEST RADIOGRAPHY  Marked bilateral apical pleural thickening  Pneumothorax HIGH-RESOLUTION CT  Intense fibrosis of the visceral pleura  Sparing of the parenchyma distant from the pleura  Traction bronchiectasis  A slight shrinking of the lung, otherwise almost equal findings for many years  There is currently no specific therapy  in progressive cases lung Tx is ultima ratio

Case of the Month 11 Further Reading Pleuroparenchymal fibroelastosis 1) Frankel SK, Cool CD, Lynch DA et-al. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004;126 (6): ) Becker CD, Gil J, Padilla ML. Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?. Mod. Pathol. 2008;21 (6): ) Piciucchi S, Tomassetti S, Casoni G et-al. High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir. Res. 2011;12 (1): 111.