Pleuroparenchymal Fibroelastosis in a Patient With Undifferentiated Connective Tissue Disease and Marked Scleroderma Features

Abstract

Introduction: We report a case of idiopathic pleuroparencymal fibroelastosis (PPFE) and bilateral pneumothoraces in a patient with undifferentiated connective tissue disease (UCTD) and marked scleroderma features. CASE PRESENTATION: A 54 year old female, never smoker, presented with a one month history of dyspnea and one week of bilateral pleuritic chest pain. Her past medical history is significant for hypertension and review of systems is positive for raynaud's phenomenon. She has no family history of autoimmune disease. On physical examination she has hypopigmented skin lesions, perioral fibrosis and bilateral upper lung field crackles. Chest imaging was performed (Figure 1). Laboratory data was remarkable for ANA greater than 1:2560 (nucleolar pattern). Antitopoisomerase I (anti-Scl-70), anticentromere, anti-Smith, anti-SSA, anti-SSB and anti-ribonucleoprotein were negative. Since she did not meet diagnostic criteria for scleroderma we consider she has UCTD. Pulmonary function studies revealed a severe restrictive defect and moderately reduced diffusing capacity. She underwent a video-assisted thoracoscopic wedge biopsy of the left upper lobe. Surgical pathology showed subpleural scarring composed of fibroelastosis within and below the pleura (Figure 2). She had a prolonged hospitalization and she required multiple chest tube placements to achieve adequate lung re-expansion. Three months after discharge her dyspnea had improved and she did not require supplemental oxygen. As there are no effective therapies for PPFE, we will refer her for lung transplant evaluation when appropriate. DISCUSSION: Idiopathic PPFE is a rare idiopathic interstitial pneumonia characterized by upper lobe predominant dense subpleural consolidations on imaging and a histology of upper lobe predominant fibroelastosis involving the pleura and subpleural lung parenchyma.1) There are multiple reported autoimmune associations with PPFE including rheumatoid arthritis, ankylosing spondylitis and psoriasis.(2) In our review we found no reports of PPFE and UCTD with scleroderma features. Conclusions: Our case describes a novel association of PPFE and UCTD with marked scleroderma features.