PLEUROPARENCHYMAL FIBROELASTOSIS UNCOVERED IN A PATIENT WITH COVID-19 PRESENTING WITH PNEUMOTHORAX

ABSTRACT

SESSION TITLE Diagnosis of Lung Disease through Pathology Case Posters SESSION TYPE Case Report Posters PRESENTED ON 10/19/2022 1245 pm - 0145 pm INTRODUCTION: This report describes the case of a patient presenting with pneumothorax and Severe Acute Respiratory Syndrome (SARS) Coronavirus-2 (SARS-cov-2) infection leading to Coronavirus Disease 2019 (COVID-19) pneumonia, with worsening presentation, later found to have underlying Pleuroparenchymal Fibroelastosis (PPFE). CASE PRESENTATION A 68 year old male with a past medical history of hypertension and type 2 diabetes presented to his primary care clinic with shortness of breath. He underwent a Chest X-Ray as an outpatient which revealed a moderate right-sided pneumothorax (PTX), and he was sent to the Emergency Department by his primary care provider. He was found to be COVID positive on initial workup, also requiring supplemental oxygen. Other routine laboratory tests did not reveal any significant abnormalities. His shortness of breath worsened and on repeat X-rays his pneumothorax increased in size therefore a chest tube was placed by Cardiothoracic Surgery. Computerized Tomography of the chest revealed moderate right pneumothorax, bilateral diffuse ground glass opacities and pulmonary micronodules [Figure 1]. The patient had mild initial improvement and the chest tube was removed but he had recurrence of the PTX and he underwent urgent Video Assisted Thoracoscopic Surgery (VATS), with right upper lobe wedge resection and talc pleurodesis. A biopsy of the resected lung revealed a benign lung with fibroelastotic scarring, diffusely involving subpleural tissue and prominently extending into and entrapping areas of underlying alveolated tissue, with no inflammation, granulomas or pneumonia noted. Workup for tuberculosis, autoimmune disorders, HIV was negative. He eventually was discharged home with close pulmonology and cardiothoracic surgery follow ups, planned for disease surveillance and malignancy workup. DISCUSSION: PPFE is a rare entity, and classified amongst rare causes of idiopathic interstitial pneumonias (IIP) [1]. It is characterized by upper lobe fibrosis, supleural and parenchymal scarring. It can occur at any age, and the usual presentation is of pneumothorax in a thin male, with a shortened anteroposterior diameter of the chest. Radiographic findings typically include subpleural nodular or reticular opacities in the upper lobes, usually sparing the middle and lower lobes. Pathology reveals increased elastic tissue and dense collagen fibers, along with subpleural fibrosis [2]. Pulmonary function testing reveals a restrictive pattern with reduced diffusion capacity and it is usually resistant to steroids [3]. CONCLUSIONS: PPFE is an uncommon cause of insidious, slowly progressive fibrotic lung disease often limited to the upper lobes. It should be suspected in any person presenting with recurrent pneumothorax or blebs without other known inciting causes. Lung biopsy helps establish the diagnosis. Patients with this condition need close pulmonology follow up to assess progression. Reference #1 Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, Behr J, Bouros D, Brown KK, Colby TV, Collard HR, Cordeiro CR, Cottin V, Crestani B, Drent M, Dudden RF, Egan J, Flaherty K, Hogaboam C, Inoue Y, Johkoh T, Kim DS, Kitaichi M, Loyd J, Martinez FJ, Myers J, Protzko S, Raghu G, Richeldi L, Sverzellati N, Swigris J, Valeyre D;ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013 Sep 15;188(6)733-48. doi 10.1164/rccm.201308-1483ST. PMID 24032382;PMCID PMC5803655. Reference #2 Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis description of a novel clinicopathologic entity. Chest. 2004 Dec;126(6)2007-13. doi 10.1378/chest.126.6.2007. PMID 1559 706. Reference #3 Watanabe K. Pleuroparenchymal Fibroelastosis Its Clinical Characteristics. Curr Respir Med Rev. 2013 Jun;9(4)299-237. doi 10.2174/1573398X0904140129125307. PMID 24578677;PMCID PMC3933942. DISCLOSURES No relevant relationships by FNU Amisha No relevant relationships by Perminder Gulani No relevant relationships by Hyomin Lim No relevant relationships by paras malik No relevant relationships by Divya Reddy