PLEUROPARENCHYMAL FIBROELASTOSIS FROM BEGINNING TO END: FOLLOWING THE DISEASE COURSE OF THIS RARE INTERSTIAL LUNG DISEASE

Abstract

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pleuroparenchymal fibroelastosis has become more recognized in the clinical literature as an entity characterized by predominantly upper lobe localization of pleural and subpleural fibrosis and elastosis. In this case report, we present a patient with idiopathic PPFE throughout the course of several hospital admissions over a two-year time period: from her initial presentation to her eventual compassionate extubation. CASE PRESENTATION: A 63 y/o Hispanic female presented with dyspnea secondary to a L pneumothorax. A thorough rheumatologic and infectious work-up was performed (Table 1). A HRCT demonstrated "tree-in bud pathology, bronchiectasis, and changes not consistent w/ UIP or NSIP.” She completed a 6-minute walk test on room air upon initial presentation, but 10 months later she developed another large pneumothorax over her LUL. She was again admitted one month later for a R pneumothorax requiring chest tube decompression. It was during this prolonged admission that a CT thorax demonstrated worsening fibrotic lung disease with pleural thickening and architectural distortion (Image 1, Image 2). Another 6-minute walk test demonstrated desaturation to 88% on room air with 1L O2 requirement upon exertion. By her 6th hospitalization, a 6-minute walk test demonstrated significant respiratory distress with exertion, despite supplemental O2. 2 years following her initial presentation, the patient was admitted for hypoxic respiratory failure, intubated in the ED and ultimately compassionately extubated. DISCUSSION: The gold-standard for diagnosis is histopathologic confirmation. This patient was referred for a lung biopsy several times, but these records could not be confirmed. Instead, at a multidisciplinary conference with Rheumatology, Pulmonology, ID, and Radiology, the patient's clinical course and imaging were reviewed and found to be most consistent with PPFE. The reported mean age of presentation of PPFE is 49, while our patient presented later in life with severe radiologic features, suggesting years of subclinical progression. Some studies have suggested that the decline of FVC in patients can be separated into two heterogeneous groups – one with decline in FVC over years and those that rapidly deteriorate. Given our patient's rapid progression over two years; she likely falls into the latter group. Her course fits with prior studies indicating a subset of patients with progressive disease may have a median survival of <5 years. CONCLUSIONS: This is a singular case report of an elderly female patient, whose disease course was captured in the advanced stage of disease. The patient was referred for transplant, but due to her functional status and low BMI, she was not deemed an appropriate candidate. Despite more recognition of this condition, further longitudinal evaluation of patients is required in order to determine the nature of the course of disease. Reference #1: Chua F, Desai SR, Nicholson AG, et al. Pleuroparenchymal fibroelastosis a review of clinical, radiological, and pathological characteristics. Ann Am Thorac Soc. 2019;16(11):1351-1359. https://doi.org/10.1513/AnnalsATS.201902-181CME Reference #2: Yoshida Y, Nagata N, Tsuruta N, et al. Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis. 2015. https://doi.org/10.1016/j.resinv.2015.11.002 Reference #3: Tanizawa K, Handa T, Kubo T, et al. Clinical significance of radiological pleuroparenchymal fibroelastosis pattern in interstitial lung disease patients registered for lung transplantation: A retrospective cohort study. Respir Res. 2018;19(1). https://doi.org/10.1186/s12931-018-0860-6 DISCLOSURES: No relevant relationships by Rodrigo Garcia Tome, source=Web Response No relevant relationships by Chongiin Kim, source=Web Response