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Diffuse Lung Disease |

Idiopathic Pleuroparenchymal Fibroelastosis (IPPFE) Associated With Undifferentiated Connective Tissue Disease (UCTD)

Noreen Murphy*, MD; Trina Hollatz, MD; Keith Meyer, MD
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University of Wisconsin, Madison, WI


Chest. 2012;142(4_MeetingAbstracts):472A. doi:10.1378/chest.1388581
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Abstract

SESSION TYPE: ILD Student/Resident Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Frankel et al. (1) reported a unique form of pleuro-parenchymal lung disease in 2004 characterized by marked pleural and parenchymal radiographic involvement with upper lobe predominance. Pathologic findings included pleural thickening with fibrosis, subpleural fibroelastosis, sparing of parenchyma distant from the pleurae, patchy lymphoplasmacytic infiltrates, elastic fiber proliferation and fibroblastic foci at the leading edge of fibrosis. Additionally, these patients were noted to be prone to the development of secondary spontaneous pneumothoraces (SSPTX). The etiology of IPPFE remains unknown. We present the case of a patient with radiologic and pathologic findings consistent with IPPFE who met criteria for UCTD (2).

CASE PRESENTATION: A 65 year-old female with a 6-year history of interstitial lung disease (ILD), psoriasis and Raynaud’s disease noted worsened dyspnea and presented for lung transplant (LTX) evaluation. She reported chronic cough productive of clear sputum and used oxygen at night and with activity. Chest auscultation revealed scattered crackles and mid-inspiratory squeaks. ANA titer was positive (1:640) with nucleolar and speckled patterns; rheumatoid factor was negative. Decreased DLCO and a restrictive ventilatory defect were present on pulmonary function testing. Chest imaging (HRCT) demonstrated upper lobe-predominant fibrosis with reticulation, severe traction bronchiectasis and honeycombing with mosaic attenuation that was greatest in the lung bases and indicative of air trapping. She was listed for LTX with the diagnosis of pulmonary fibrosis; while listed, she developed a SSPTX. She received a single LTX 2 months later. Histopathologic examination of the explanted lung revealed extensive pleuro-parenchymal fibrosis and elastosis with prominent peribronchial and perivascular fibroelastic proliferation and honeycomb change with fibroelastic foci in the interstitium.

DISCUSSION: Frankel et al. labeled this disease as IPPFE (1). Since their initial report of 5 patients, 9 additional cases have been reported. We present a case of IPPFE in a patient with UCTD. None of the 14 patients reported in the literature had positive autoimmune serologies or evidence of CTD, but 4 were bone marrow transplant recipients, which suggests that immunologic factors may play a role in the development of IPPFE (3).

CONCLUSIONS: We suggest this patient’s systemic autoimmune process may have played a role in the pathogenesis of this unique pattern of lung injury and fibrosis.

1) Frankel SK et al. Chest. 2004;126(6):2007-13.

2) Kinder BW, et al. Am J Respir Crit Care Med 2007(7);176;491-7.

3) von der Thüsen JH et al. Mod Pathol. 2011;24(12):1633-9.

DISCLOSURE: The following authors have nothing to disclose: Noreen Murphy, Trina Hollatz, Keith Meyer

No Product/Research Disclosure Information

University of Wisconsin, Madison, WI

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