SESSION TITLE: Interstitial Lung Disease Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 26, 2014 at 03:15 PM - 04:15 PM
INTRODUCTION: Pleuroparenchymal fibroelastosis (PPFE) is a rare form of idiopathic interstitial pneumonia (IIP) recently recognized by the American Thoracic Society as a distinct clinical entity. The underlying etiology and pathophysiology of this disease is not yet fully understood but has been reported in several patients who have undergone allogeneic bone marrow transplant (BMT)1. Here we present a case of PPFE in a patient who received an autologous stem cell transplant (SCT).
CASE PRESENTATION: Our patient is a 64 year old male nonsmoker with a history of mantle cell lymphoma who underwent an autologous SCT in 1998. The transplant was succesful and he remained in good health until late 2013 when he presented with chest pain and dyspnea. He also noted recurrent episodes of respiratory tract infections requiring antibiotics at least four times a year over the past ten years. A chest radiograph showed a spontaneous right apical pneumothorax followed by a chest CT notable for a moderate sized right pneumothorax and visceral pleural thickening with predominance in the upper lung zones bilaterally, and lower lung zone scattered reticulations, septal fibrosis, and traction bronchiectasis. In retrospect, these changes had been developing over the past decade. An open lung biosy was performed; the specimen obtained from the RUL showed subpleural fibrosis and fibroelastosis while that taken from the RLL showed subpleural fibrosis with fibroblastic foci and areas of honeycombing. Based on history, imaging, and pathology, the diagnosis of PPFE has been made.
DISCUSSION: PPFE is a rare, newly recognized form of IIP without a clear etiology. There have been case reports in patients who have had allogeneic BMT; all presented at various points after transplant with pneumothoraces and recurrent infections1. There is the hypothesis that PPFE may represent a form of graft-vs-host disease, however, our patient received an autologous SCT, making GVHD less likely. It is also possible that another autoimmune process related to BMT or recurrent infections in a susceptible individual may lead to PPFE.
CONCLUSIONS: We believe that this case of PPFE after autologous SCT is only the second such case to be reported and is an important diagnosis to keep in the differential when evaluating this patient population, even if it has been many years since transplant.
Reference #1: von der Thüsen, JH, et al. Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Modern Pathology (2011) 24, 1633-1639
DISCLOSURE: The following authors have nothing to disclose: Kristyn Sayball, Kristin Miller, Margaret Grimes, Harold Chung
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