PLEUROPARENCHYMAL FIBROELASTOSIS: A RARE INTERSTITIAL LUNG DISEASE

Abstract

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD) characterized by upper lobe predominant pleural and parenchymal fibrosis. We report a patient with clinical and radiographic findings consistent with PPFE with no other explanation for her ILD. CASE PRESENTATION: A 65 year-old female with history of depression, hypothyroidism, and irritable bowel syndrome presented to the pulmonary clinic with chronic dry cough and exertional dyspnea. She also endorsed malaise and fatigue, but denied orthopnea, paroxysmal nocturnal dyspnea, chest pain, or any other symptoms. Physical exam was only notable for a few scattered fine crackles at the upper lung fields. Pulmonary Function Tests revealed a mildly reduced DLCO with no other abnormalities. A high resolution CT scan (HRCT) of the chest performed due to clinical suspicion for interstitial lung disease demonstrated bi-apical consolidative-type changes upper lobe volume loss and peripherally-based reticular opacities (also involving the superior segments of the lower lobes). A transthoracic echocardiogram showed normal left and right ventricular size and function. Our patient did not have any occupational or medication exposures. An extensive rheumatological workup was negative. The patient declined lung biopsy. Given the patient's progressive pulmonary symptoms, the decision was made to initiate antifibrotic therapy with pirfenidone, as the patients' irritable bowel syndrome with diarrhea precluded her from nintedanib therapy. DISCUSSION: PPFE is a chronic, progressive lung fibrotic disorder with unclear etiology, but may be associated with chemotherapy, lung or bone marrow transplantation, or recurrent pulmonary infections. Symptoms include dry cough, dyspnea, weight loss or pneumothorax. A thorough history and evaluation is critical to exclude other potential interstitial lung disorders. Platythorax, a reduction of the anterior-posterior diameter of the thorax relative to the transverse diameter, has been shown to be a clinical sign and a potential marker of disease progression. HRCT typically shows bilateral apical pleural thickening with architectural distortion, dense subpleural consolidation and traction bronchiectasis, with upper lobe volume loss. Definitive diagnosis is established by surgical lung biopsy. There is presently no approved therapy for PPFE, although there is limited evidence to support the use of antifibrotic agents. CONCLUSIONS: Given the rarity of PPFE, it is important for providers to recognize the existence of this lung condition as it has both prognostic and therapeutic implications. REFERENCE #1: Chua, Felix. Annals of the American Thoracic Society, 30 May 2019, www.atsjournals.org/doi/full/10.1513/AnnalsATS.201902-181CME. REFERENCE #2: Reddy, Taryn L., et al. "Pleuroparenchymal Fibroelastosis: a Spectrum of Histopathological and Imaging Phenotypes." European Respiratory Society, European Respiratory Society, 1 Aug. 2012, erj.ersjournals.com/content/40/2/377. REFERENCE #3: Redondo, M.T., et al. "Idiopathic Pleuroparenchymal Fibroelastosis: A Rare but Increasingly Recognized Entity." Pulmonology, Elsevier, 1 Jan. 2015, www.journalpulmonology.org/pt-idiopathic-pleuroparenchymal-fibroelastosis-a-rare-articulo-X0873215915856150. DISCLOSURES: No relevant relationships by Raymond Foley, source=Web Response No relevant relationships by Simrina Sabharwal, source=Web Response No relevant relationships by Evan Wasserman, source=Web Response