Case report Idiopathic pleuroparenchymal fibroelastosis e A rare idiopathic interstitial pneumonia Balamugesh Thangakunam a , Barney T. J. Isaac a, * , Devasahayam Jesudas Christopher a , Deepak Burad b a Department of Pulmonary Medicine, Christian Medical College, Vellore, India b Department of General Pathology, Christian Medical College, Vellore, India article info Article history: Received 9 October 2015 Received in revised form 17 November 2015 Accepted 18 November 2015 Keywords: Interstitial lung disease Idiopathic interstitial pneumonia Pleuroparenchymal fibroelastosis abstract Idiopathic pleuroparenchymal fibroelastosis is a rare idiopathic interstitial pneumonia. It was first described in 2004 and subsequently included in the ATS/ERS classification of idiopathic interstitial pneumonia in 2013. There have been few cases reported so far. The diagnostic criteria is still emerging and its etiology is being questioned. We report a case of pleuroparenchymal fibroelastosis probably idiopathic, the first of its kind to be reported from India, and a brief review of the literature. © 2015 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). 1. Introduction Idiopathic pleuroparenchymal fibroelastosis is a chronic idio- pathic interstitial pneumonia, characterised by upper lobe pre- dominance, significant involvement of the pleura and a histopathology revealing subpleural fibroelastosis. It was first described in 2004 and subsequently included in the ATS/ERS clas- sification of Idiopathic Interstitial Pneumonia (IIP) in 2013 under the category of rare IIP's. Sporadic cases and few series have been reported from all across the globe, but none of these have been from India. We report a case of pleuroparenchymal fibroelastosis probably idiopathic, the first of its kind from India. 2. Case description A 41 year old lady presented with dry cough and progressive breathlessness for a year. She was evaluated in another centre with a CT thorax and VATS biopsy 3 months into illness. This showed fibrosing interstitial pneumonia (the slides reviewed in our centre was reported as organizing pneumonia). Post biopsy, she developed a left sided loculated pneumothorax, and this remained even after intercostal drainage. She was treated with oral corticosteroids. Af- ter a brief period of stability, her symptoms continued to progress. Azathioprine was added on for another 3 months with no response and hence stopped. She was then briefly initiated on Pirfenidone, which was stopped due to intolerance. When she presented to us, she was only on oral steroids. In the mean time, she developed a spontaneous pneumothorax on right side which completely resolved with intercostal tube drainage. There was no history suggestive of a connective tissue disorder. Her co-morbidities were type 2 diabetes mellitus, hypothyroidism and primary infertility. On examination, she was not clubbed. She had reduced air entry in the left infraclavicular region and bilateral diffuse crackles and squeaks. Her room air saturation was 95% but she quickly desatu- rated to 82% on exertion. Her ABG revealed chronic type 2 respi- ratory failure with hypoxia. Her CT scan (Fig. 1) revealed loculated pneumothorax in left upper zone, pleural and subpleural paren- chymal fibrosis with upper lobe predominance. The fibrotic changes have significantly worsened compare to previous CT picture. The differentials considered were idiopathic pulmonary fibrosis, idiopathic pleuroparenchymal fibroelastosis and chronic hyper- sensitivity pneumonitis. Absence of response to steroids and absence of triggering factors dissuaded a diganosis of chronic hy- persensitivity pneumonitis. She was started on the maximum tolerable dose of Pirfenidone(400 mg thrice a day) and the oral * Corresponding author. Department of Pulmonary Medicine, Christian Medical College, Vellore 632004, Tamilnadu, India. E-mail addresses: drbalamugesh@yahoo.com (B. Thangakunam), barneyisaac98@gmail.com (B.T.J. Isaac), djchris@cmcvellore.ac.in (D.J. Christopher), deepakburad@cmcvellore.ac.in (D. Burad). Contents lists available at ScienceDirect Respiratory Medicine Case Reports journal homepage: www.elsevier.com/locate/rmcr http://dx.doi.org/10.1016/j.rmcr.2015.11.004 2213-0071/© 2015 The Authors. Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Respiratory Medicine Case Reports 17 (2016) 8e11