Histological evolution of pleuroparenchymal fibroelastosis

Abstract

AimsTo investigate the histological evolution in the development of pleuroparenchymal fibroelastosis (PPFE).Methods and resultsWe examined four patients who had undergone surgical lung biopsy twice, or who had undergone surgical lung biopsy and had been autopsied, and in whom the histological diagnosis of the first biopsy was not PPFE, but the diagnosis of the second biopsy or of the autopsy was PPFE. The histological patterns of the first biopsy were cellular and fibrotic interstitial pneumonia, cellular interstitial pneumonia (CIP) with organizing pneumonia, CIP with granulomas and acute lung injury in cases 1, 2, 3, and 4, respectively. Septal elastosis was already present in the non-specific interstitial pneumonia-like histology of case 1, but a few additional years were necessary to reach consolidated subpleural fibroelastosis. In case 3, subpleural fibroelastosis was already present in the first biopsy, but only to a small extent. Twelve years later, it was replaced by a long band of fibroelastosis. The septal inflammation and fibrosis and airspace organization observed in the first biopsies were replaced by less cellular subpleural fibroelastosis within 3–12 years.ConclusionsInterstitial inflammation or acute lung injury may be an initial step in the development of PPFE.

Pleuroparenchymal Fibroelastosis of the Lung: A Review

“…7 Its pathogenesis remains unclear, although it is proposed that the starting point may involve an element of acute lung injury or interstitial inflammation. 8 In fact, areas of diffuse alveolar damage in patients with restrictive allograft syndrome after lung transplantation with ensuing PPFE appear to support this hypothesis. 15 Nonetheless, whatever the pathogenesis may be, it remains clear that once PPFE pattern is identified, 3,5 the disease is often rapidly progressive, with frequent complication of pneumothorax and poor prognosis.…”

“…Subsequently, von der Thüsen 3 summarized the information gleaned from properly documented case series, which were mostly from the English literature. With that information as well as information from recent publications, 5,7,8 there have been about 120 cases reported in the world literature. The age range of the patients is from 13 to 87 years.…”

“…In problematic cases, 3 performance elastic fiber stain, such as elastic Van Gieson stain, would be useful because the stain demonstrates a more sparse, fragmented, and disorganized elastic fiber distribution in the areas of fibrosis in UIP 4 compared with more marked and established elastic fiber deposition in relation to the alveolar walls in PPFE, which is described as containing at least twice as much elastin compared with UIP. 8 Recently, lesion with combined patterns of UIP and PPFE has been noted; in such an instance Oda et al 11 proposed labeling the lesion as PPFE, and this entity with combined patterns is associated with a much more dismal outcome compared with either idiopathic pulmonary fibrosis or PPFE alone.…”

Unilateral Upper Lung Field Pulmonary Fibrosis Radiologically Consistent with Pleuroparenchymal Fibroelastosis after Thoracotomy: A New Disease Entity Related to Thoracotomy

“…Therefore, mechanisms other than an impaired thoracic movement should also contribute to the development of unilateral Upper-PF. We believe that the clinical courses of an apical cap in the nonoperated side, which was observed during the first visit in [2,12] , we believe that our 3 patients with a history of gastroesophageal resection potentially had a mechanism of microaspiration, which resulted in repeated low-grade inflammation. Generally, gastroesophageal reflux disease (GERD) and delayed gastric emptying easily develop in patients with a history of gastrectomy or esophagectomy [15][16][17] and can cause aspiration pneumonia [18] .…”

“…These results imply that the presence of an apical cap in the operated side is particularly important for unilateral Upper-PF development. More recently, apical cap, which is pathologically identical to PPFE, has been reported to be potentially caused by relative ischemia in the upper lobe and low-grade inflammation in the lung parenchyma [2,12] . Besides, relative ischemia in the upper lobe can impair the resolution of inflammation, which would also facilitate the development of apical cap and PPFE [4,13,14] .…”