Idiopathic Pleuropulmonary Fibroelastosis: Fact or Fancy?
Xiaofang Yang, Parnian Ahmadi Moghaddam, Ali Akalin, Armando Fraire. UMass Memorial Medical Center, Worcester, MA; Contribute Equally, UMASS Medical Center, Worcester, MA
Background: Criteria for the diagnosis of Idiopathic Pleuropulmonary Fibroelastosis (IPPFE) have been recently formulated. Proposed basic criteria for diagnosis include upper lobe predominance, dense interstitial intraalveolar fibrosis, prominent elastosis, dense fibrotic thickening of the overlying pleura and by definition no known cause. These features however are all non-specific and may be seen in other conditions such as advanced sarcoidosis, drug or radiation induced fibrosis, connective tissue disorders and asbestosis.
Design: We recently studied one case of possible IPPFE. The patient, an 85 year old woman, had thick white fibrotic patches in both the RUL and the LUL of the lung. Microscopically, the pleura had extensive fibrosis with elastotic changes of the subjacent pulmonary parenchyma. A search of our files was conducted and identified 20 additional cases with localized areas of pleuropulmonary fibroelastosis. Eleven of the 21 cases were men and 10 were women. Their ages ranged from 32 to 85 with a median of 67 years. Radiologically (CXR and CT), 7 had pneumothorax, 3 had emphysema, 3 had both pneumothorax and emphysema, and 6 had nodular lung lesions including 3 carcinomas, 1 hamartoma, and 1 old infarct. Some patients had more than 1 lesion identified and three patients had no imaging studies available.
Results: Grossly identifiable pathologic changes were identified in all 21 cases. These changes included emphysema, focal areas of lung consolidation, white pleural patches, subpleural nodularities, bullae, blebs and in one case a cavitary lesion. Nine cases were distinctly apical in location and 3 were both apical and lower lobe. A precise location was not determined in the remaining nine cases. Elastosis and /or fibroelastosis of varying degree were microscopically observed in all 21 cases. Our index case, the 85 year old woman, had resolving pneumonia at autopsy.
Conclusions: IPPFE has been described as a distinct clinicopathological entity with a striking upper lobe predilection and no known cause. Our study disclosed a wide variety of associated conditions, most frequently underlying pneumothorax and emphysema, with or without bullous change. These findings suggest that at least in our case material, IPPFE is commonly a localized phenomenon, likely secondary to a wide variety of associated post pneumonic or post pleuritic injuries. None of our cases appeared to be truly idiopathic.
Tuesday, March 5, 2013 1:00 PM
Poster Session IV # 290, Tuesday Afternoon