Radiologic pleuroparenchymal fibroelastosis-like lesion in connective tissue disease-related interstitial lung disease

Abstract

BackgroundRadiologic pleuroparenchymal fibroelastosis (PPFE)-like lesion including pulmonary apical cap can be occasionally observed in clinical settings. However, the significance of radiologic PPFE-like lesion is unclear in connective tissue disease (CTD)-related interstitial lung disease (ILD).Materials and methodsA total of 113 patients with CTD-related ILD were enrolled and assessed for radiologic PPFE-like lesion, which was defined as bilateral, upper lobe, and subpleural dense consolidations with or without pleural thickening on chest high-resolution computed tomography. The clinical, radiologic, and pathologic characteristics were evaluated.ResultsRadiologic PPFE-like lesion was found in 21 patients (19%) and were relatively frequent in those with systemic sclerosis (6/14: 43%) and primary Sjögren's syndrome (4/14: 29%). Patients with PPFE-like lesion were significantly older, had lower body mass index, higher ratio of residual volume to total lung capacity, and higher complication rate of pneumothorax and/or pneumomediastinum than those without. Twelve of the 21 patients were diagnosed radiologically as usual interstitial pneumonia (UIP) or possible UIP pattern. Two of three patients who underwent surgical lung biopsy of the upper lobes showed UIP on histopathology. Another patient was confirmed to have upper lobe PPFE on autopsy. During the clinical course, progression of the radiologic PPFE-like lesions was observed in 13 of 21 patients. Six patients died (mortality rate: 29%) and their PPFE-like lesions were commonly progressive. In the total cohort, our multivariate analysis identified the presence of PPFE-like lesion as a significant risk factor for respiratory death (hazard ratio: 4.10, 95% confidence interval: 1.33–12.65, p = 0.01).ConclusionIn patients with CTD-related ILD, radiologic PPFE-like lesion, which may present as not only PPFE but also apical cap and upper lobe subpleural fibrosis commonly due to UIP, was not uncommon and was associated with poor prognosis. Clinicians should be cautious with this radiologic finding, particularly when it is progressive.

Podoplanin‐positive myofibroblasts: a pathological hallmark of pleuroparenchymal fibroelastosis

“…Both radiologically and pathologically, it is difficult to distinguish PPFE from PAC, particularly at a single time-point. 4,14 The present study emphasised this difficulty further, even using immunohistochemistry. Interestingly, Sekine et al reported recently that lobectomy for lungs with PAC might be a risk for the development of PPFE, 15 suggesting the potential of PAC as a pre-PPFE lesion.…”

“…Both radiologically and pathologically, it is difficult to distinguish PPFE from PAC, particularly at a single time‐point . The present study emphasised this difficulty further, even using immunohistochemistry.…”

Clinical diagnosis of idiopathic pleuroparenchymal fibroelastosis: A retrospective multicenter study

“…However, there was no prognostic difference between patients with and without the lower lobe UIP/possible UIP pattern, suggesting that the presence of a lower lobe lesion may not be so critical to the prognosis of idiopathic PPFE. Conversely, we have previously reported that the presence of PPFE or PPFE-like lesions, as an additional finding, can affect the clinical course and worsen the prognosis in the background of IPF or connective tissue disease-related ILD [17,18]. Collectively, PPFE may be a poor prognostic determinant, regardless whether it represents a major pattern of ILD or an additional pathologic/radiologic finding.…”

“…The differential diagnoses for idiopathic PPFE include IPF, chronic hypersensitivity pneumonitis, pulmonary sarcoidosis, pneumoconiosis, connective tissue disease-related ILD, and pulmonary infections [6,18,19]. Even with a careful medical interview, systemic screening, and rigorous radiologic assessment, it may be occasionally difficult to exclude these diseases without pathologic evaluation.…”

Major lung complications of systemic sclerosis

“…For example, fibroelastosis is increasingly being recognized as a feature of connective tissue disease-associated ILD and can occur in association with pleural abnormalities and resembles idiopathics pleuroparenchymal fibroelastosis (a rare form of ILD with a poor prognosis). This form of ILD might be more progressive than other forms of SSc-associated ILD [97]. Currently, the precise frequency and clinical relevance of fibroelastosis in SSc-associated ILD is unknown [98].…”