Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?

Abstract

Idiopathic pleuroparenchymal fibroelastosis is a rare recently described entity likely to be under-and misdiagnosed, as awareness of this entity is not yet widespread. We report two cases that show the need to include this disease in the differential diagnosis of patients with predominantly pleural and subpleural fibrotic processes. The condition is a fibrotic thickening of the pleura and subpleural parenchyma due to elastic fiber proliferation predominantly in the upper lobes. Performing elastic fiber stains routinely in patients with fibrosis of this distribution may, therefore, aid in establishing the diagnosis and differentiating it from usual interstitial pneumonia/idiopathic pulmonary fibrosis. These patients may be prone to the development of secondary spontaneous pneumothoraces and persistent postoperative bronchopleural fistulae. Continued study of newly diagnosed cases may uncover shared characteristics or features helpful in generating an etiologic hypothesis. Only with better understanding of this disease can we hope in the future to be able to offer treatments other than supportive care and ultimately lung transplantation, which are the only therapeutic options available today.

Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation

“…11 This histological pattern of interstitial fibrosis has hitherto only been reported in an idiopathic setting with the seven patients being adults (average age, 51 years) and six being female. 12,13 As is the case in our patients, in those series, pleuroparenchymal fibroelastosis was found to predispose to secondary spontaneous or iatrogenic pneumothoraces. The first series included a patient with a sibling who had died of progressive pleuroparenchymal fibroelastosis, and two patients who had been treated with chemotherapy, but lacked a potentially unifying causal factor.…”

Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis

“…It is characterized radiologically by features suggestive of a chronic interstitial pneumonia with upper lobe predominance, and histologically by pleural fibrosis and parenchymal fibroelastosis in a predominantly subpleural distribution, with a sharp demarcation between fibroelastotic and unaffected lung parenchyma, and with the presence of fibroblastic foci at this interface. A limited number of cases with similar radiologic and pathologic features have also been reported, [8][9][10][11][12][13][14] including a very recent article by Reddy et al, 14 suggesting a broader spectrum of histopathologic findings.…”

“…On the other hand, the presence of concurrent diffuse alveolar damage in a large proportion of restrictive allograft syndromepleuroparenchymal fibroelastosis cases may also suggest that pleuroparenchymal fibroelastosis represents a more non-specific late sequela of diffuse alveolar damage, which would be in keeping with the suggestion made by von der Thusen et al that pleuroparenchymal fibroelastosis in post-bone marrow transplant patients may represent a late complication of post-bone marrow transplant idiopathic pneumonia syndrome, which has a high mortality rate and is characterized clinically and radiologically by features consistent with diffuse alveolar damage. 15 If so, pleuroparenchymal fibroelastosis may represent a late complication of multiple and varied etiologic factors that result in acute lung injury/diffuse alveolar damage, including chemo/radiotherapy 7,10,11,15 and infections. 14 Finally, the finding of acute and/or organizing thromboemboli in our series raises the question of the role of vascular injury in the pathophysiology of pleuroparenchymal fibroelastosis.…”

“…[8][9][10][11][12][13][14] Radiologically, idiopathic pleuroparenchymal fibroelastosis has been characterized by pleural thickening associated with underlying parenchymal reticular abnormalities, architectural distortion and traction bronchiectasis in a mostly upper lobepredominant distribution. 7,10,11 Histologically, idiopathic pleuroparenchymal fibroelastosis is characterized by pronounced visceral pleural fibrosis associated with subpleural, 'intra-alveolar' fibrotic changes characterized by an admixture of elastic and fibrous tissue (fibroelastosis).…”

Pleuroparenchymal fibroelastosis from a consecutive database: a rare disease entity?

“…The 14 cases were re-evaluated in multidisciplinary discussions using histopathological and radiological criteria for the diagnosis of PPFE [1,2,5]. Two cases were excluded from a diagnosis of PPFE; one did not fulfil the radiological criteria and the other showed a pathological pattern of usual interstitial pneumonia (UIP), with only focal elastosis.…”