PLEUROPARENCHYMAL FIBROELASTOSIS: A RARE CASE OF INTERSTITIAL LUNG DISEASE AND PNEUMOTHORACES

Abstract

SESSION TITLE: Diffuse Lung Disease 2 SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/07/2018 04:45 pm - 05:45 pm INTRODUCTION: In 1992, an interstitial lung disease with upper lobe predominant fibrosis was first described in Japan; approximately 20 years later, the disease entity was coined as Pleuroparenchymal Fibroelastosis (PPFE). To date, there have been approximately 120 cases reported world-wide in the literature. The most common presenting complaints are shortness of breath, significant weight loss, pneumothorax, or chest pain. The pathogenesis remains unclear and the disease is often rapidly progressive with a poor prognosis. CASE PRESENTATION: We present a 65 year old female with a history of interstitial lung disease complicated by multiple pneumothoraces and GERD was admitted to our hospital with worsening shortness of breath. The patient’s history was significant for three years of profound weight loss (BMI 14), chest pain, and shortness of breath at rest and with exertion that were unresponsive to prednisone. The patient had previously worked in a many different factories, but was a lifetime non-smoker and had no other known exposure history. The physical exam was significant for a seven liter oxygen requirement, cachexia, decreased breath sounds at the apices and diffuse fine dry crackles, without skin or nail changes. CT chest revealed changes of interstitial lung disease with septal thickening and cystic changes, as well as traction bronchiectasis and ground glass opacities with upper lobe predominance and subpleural distribution, as well as bilateral apical pneumothoraces. Pulmonary function testing was consistent with a severe restrictive defect, while autoimmune and infectious work-up were unrevealing. The decision was made to perform an open lung biopsy for definitive diagnosis, which revealed features consistent with idiopathic PPFE. DISCUSSION: Idiopathic PPFE is a rare fibrosing lung disease with an upper lobe predilection that has recently been described as a distinct clinicopathological entity with definite radiological and pathological characteristics. Due in part to the heterogeneous spectrum of clinical presentation, course, and overlap with other interstitial lung disease, it remains difficult to diagnosis and lung transplantation remains the only definitive treatment. As was the case for our patient, open lung biopsy can be crucial for diagnosis and typically reveals fibrous thickening of the visceral pleura and homogeneous, dense intra-alveolar fibrosis with septal elastosis, sharply separated from the adjacent lung parenchyma. CONCLUSIONS: PPFE should be considered in the differential diagnosis of interstitial lung diseases, especially in cases where imaging reveals subpleural fibrosis and upper lobe predominance or in patients with a history of pneumothorax, as was the case with our patient. Raising awareness of this disease entity will be crucial in the future, as the prognosis is poor and early referral for lung transplantation remains the only definitive treatment. Reference #1: English JC, Mayo JR, Levy R, Yee J, Leslie KO. Pleuroparenchymal fibroelastosis: a rare interstitial lung disease. Respirology Case Reports. 2015;3(2):82-84. https://doi.org/10.1002/rcr2.108. Reference #2: Bonifazi M, Montero MA, Renzoni EA. Idiopathic Pleuroparenchymal Fibroelastosis. Current Pulmonology Reports. 2017;6(1):9-15. https://doi.org/10.1007/s13665-017-0160-5. Reference #3: Hong SK, Khoon C, Chuah L. Pleuroparenchymal Fibroelastosis: A Review. Archives of Pathology & Laboratory Medicine. 2016;140(8):849-853 DISCLOSURES: No relevant relationships by Vijayalakshmi Ananthanarayanan, source=Web Response No relevant relationships by Aaron Muhlbauer, source=Web Response No relevant relationships by Katie Young, source=Web Response