Lower‐lobe predominant pleuroparenchymal fibroelastosis

Abstract

Upper‐lobe predominance of elastofibrosis is agreed upon for the diagnosis of clinical pleuroparenchymal fibroelastosis (PPFE). We herein describe a patient with dermatomyositis‐related interstitial pneumonia with a histology of lower‐lobe predominant PPFE. A 71‐year‐old woman who had been diagnosed with dermatomyositis‐related interstitial pneumonia died of respiratory failure. The computed tomography patterns of the lower lobes showed reticular and ground‐glass opacities with traction bronchiectasis. An autopsy revealed that the bilateral lower lobes were sclerotic with decreased air volume. A microscopic examination of the lower lobes showed pleural fibrosis and subpleural elastofibrosis without the structural destruction, indicative of histological PPFE. PPFE histology was also evident in the upper lobes but relatively modest compared to that of the lower lobes. In addition, because the computed tomography images of the patient were suggestive of non‐PPFE‐type fibrosis, lower‐lobe dominant PPFE might be overlooked in daily practice.

Remodeling of the pulmonary artery in idiopathic pleuroparenchymal fibroelastosis

“…PH has been found in 19% of IPF patients with a mildly to moderately restricted respiratory function 20 and in 38-46% of those with advanced stage disease 21,22 . Although the prevalence of PH in IPPFE patients has not been investigated, there are a few case reports of PPFE patients with PH [23][24][25] . In this cohort, we found that four of 16 IPPFE patients (25%) were complicated with clinical PH.…”

Pulmonary Apical Cap as a Potential Risk Factor for Pleuroparenchymal Fibroelastosis

“…6,7 Differentiating PPFE from PAC may be challenging, given the similar histopathologic findings (Table 1). [6][7][8][9][10][11][12][13][14][15][16][17] A history of an underlying clinical condition associated with PPFE may influence the diagnosis. Likewise, PPFE is also suggested by the presence of atypical imaging features, exceeding the expected size of an apical cap (mean, 2.2 cm), 18 or secondary histologic findings of organizing airspace disease and small airway inflammation.…”

Pleuroparenchymal Fibroelastosis: A Review with a Focus on a Non-Infectious Complications after Hematopoietic Stem Cell Transplant

“…Although initially described as an upper lobe disease, the lower lobes can also be involved in PPFE, but less commonly [ 4 , 9 , 11 , 23 ]. When lower lobes are involved, an equal distribution between the upper and lower lobes can be observed in up to one third of the patients [ 24 ]. The radiologic pattern in the lower lobes can be similar to that in the upper lobes, with pleural thickening and fibrous bands extending toward the hilum; however, findings in the lower lobes may also show diffuse interstitial thickening, suggestive of a concomitant interstitial lung disease [ 4 , 7 , 8 , 11 , 13 , 16 ].…”

“…A diagnosis of PPFE carries an overall poor prognosis, with 5-year survival rates of only 23.3–58.9% [ 7 , 8 , 14 , 24 ]. Two clinical scenarios have been described in the course of PPFE, that of a slow progression of disease and one with a more precipitous decline, with the former being the typical clinical course [ 4 , 30 ].…”