A PUZZLING CASE OF RECURRENT PNEUMONIA: PLEUROPARENCHYMAL FIBROELASTOSIS

TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Pleuroparenchymal fibroelastosis (PPFE) is a fibroelastic process mainly affecting the visceral pleura and subpleural spaces with propensity towards upper lobes. It has unique clinical, pathological radiological features1. CASE PRESENTATION: An 80 year old female history of recurrent pneumonias remote granulomatous disease presented progressive dyspnea fatigue. She denied other symptoms including chronic cough or sputum production fever chills. Chest X-ray showed extensive parenchymal pleural fibrotic changes Pulmonary function tests suggested restrictive pattern reduced diffusion capacity (DLCO). Echocardiogram no significant abnormalities. Serial CT scans chest done over two years worsening pleuroparenchymal lobes in very consistent PPFE, evidence honeycombing. This was likely idiopathic but potentially related to prior felt less be her minimal parakeet exposure associated hypersensitivity pneumonitis. Her ANA titers were mildly elevated at 1:160 further workup for auto-antibodies negative RF CCP antibodies. ESR CRP minimally elevated. A bronchoscopy offered evaluate infectious etiologies she opted wait. findings however PPFE provisional diagnosis same made. DISCUSSION: rare under-diagnosed type interstitial lung disease. mostly nature sometimes occurs as late complication after transplantation. Although similar may described patients autoimmune connective tissue disease, it usually presents certain pneumonitis, ones are best classified PPFE1. seen aged 40 70 without cough. differentiated from lobe diseases like pneumonia, sarcoidosis, non-tuberculous mycobacterial infection, pneumoconiosis, malignancy apical cap. High resolution scan remains diagnostic modality show diffuse predominantly parenchyma1. test shows DLCO. No specific laboratory diagnostic2. Surgical biopsies although not required can demonstrate intra-alveolar fibrosis elastosis2. CONCLUSIONS: The prognosis poor treatment proven effective except therapeutic transplantation1. Further studies understand pathogenesis, markers modalities this much warranted. REFERENCE #1: Chua F, Desai SR, Nicholson AG, et al. fibroelastosis. review characteristics. Ann Am Thorac Soc 2019;16:1351-9. #2: Reddy TL, Tominaga M, Hansell DM, von der Thusen J, Rassl D, Parfrey H, Guy S, Twentyman O, Rice A, Maher TM, Renzoni EA, Wells AU, AG. fibroelastosis: spectrum histopathological imaging phenotypes. Eur Respir J. 2012 Aug;40(2):377-85. DISCLOSURES: relevant relationships by Ansaam Daoud, source=Web Response Palak Rath, Lokesh Venkateshaiah,