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Differential Diagnosis of Pleuroparenchymal Fibroelastosis and Treatment Response
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A1623 - Differential Diagnosis of Pleuroparenchymal Fibroelastosis and Treatment Response
Author Block: T. Oda, E. Hagiwara, H. Kitamura, S. Ikeda, H. Nakagawa, T. Niwa, K. Isomoto, G. Matama, T. Ogura; Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
Rationale: Pleuroparenchymal fibroelastosis (PPFE) is characterized by bilateral, subpleural dense consolidation with upper lobe predominance, and upper lobe volume loss. Differential diagnosis of PPFE have not been well evaluated. Responses to treatment and outcomes of these patients are also unknown. The aim of this study was to investigate the relative frequencies of causes of PPFE-like lesion on computed tomography (CT) scan, and their differences of treatment response.
Methods: We reviewed the medical records of all 1197 diffuse lung diseases patients with surgical lung biopsy, CT-guided lung biopsy, or autopsy in our institution between 1995 and 2016. Patients were selected according to the radiologic criteria for the diagnosis of PPFE. Radiologic criteria were defined as bilateral, upper lobe, and subpleural dense consolidation with involvement of the lower lobes being absent or less marked on CT. The final diagnosis was performed by multidisciplinary discussion among radiologists, pathologists, and pulmonologists.
Results: Fifty-five of the 1197 consecutive patients met the radiologic criteria for PPFE. Of these, 32 patients fulfilled the histologic criteria for PPFE, and eight patients had chronic hypersensitive pneumonitis (CHP). Of the remaining 15 patients, four patients had unclassifiable interstitial pneumonia, three with Sjögren’s syndrome, two each with idiopathic pulmonary fibrosis, metal lung, and positive IgG4 serology, and one each with pneumoconiosis and with interstitial pneumonia with autoimmune features. Of the fifty-five patients, 22 patients received the treatment including steroid, immunosuppressant, and anti-fibrotic agent chosen at the discretion of attending physicians. Of these, one each with metal lung and Sjögren’s syndrome were able to be stabilized. The remaining 20 patients had deteriorating diseases. No patients showed improvement.
Conclusions: Differential diagnosis of PPFE are various kind of diseases. Only one each patient with metal lung and Sjögren’s syndrome were able to be stabilized.
Author Block: T. Oda, E. Hagiwara, H. Kitamura, S. Ikeda, H. Nakagawa, T. Niwa, K. Isomoto, G. Matama, T. Ogura; Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
Rationale: Pleuroparenchymal fibroelastosis (PPFE) is characterized by bilateral, subpleural dense consolidation with upper lobe predominance, and upper lobe volume loss. Differential diagnosis of PPFE have not been well evaluated. Responses to treatment and outcomes of these patients are also unknown. The aim of this study was to investigate the relative frequencies of causes of PPFE-like lesion on computed tomography (CT) scan, and their differences of treatment response.
Methods: We reviewed the medical records of all 1197 diffuse lung diseases patients with surgical lung biopsy, CT-guided lung biopsy, or autopsy in our institution between 1995 and 2016. Patients were selected according to the radiologic criteria for the diagnosis of PPFE. Radiologic criteria were defined as bilateral, upper lobe, and subpleural dense consolidation with involvement of the lower lobes being absent or less marked on CT. The final diagnosis was performed by multidisciplinary discussion among radiologists, pathologists, and pulmonologists.
Results: Fifty-five of the 1197 consecutive patients met the radiologic criteria for PPFE. Of these, 32 patients fulfilled the histologic criteria for PPFE, and eight patients had chronic hypersensitive pneumonitis (CHP). Of the remaining 15 patients, four patients had unclassifiable interstitial pneumonia, three with Sjögren’s syndrome, two each with idiopathic pulmonary fibrosis, metal lung, and positive IgG4 serology, and one each with pneumoconiosis and with interstitial pneumonia with autoimmune features. Of the fifty-five patients, 22 patients received the treatment including steroid, immunosuppressant, and anti-fibrotic agent chosen at the discretion of attending physicians. Of these, one each with metal lung and Sjögren’s syndrome were able to be stabilized. The remaining 20 patients had deteriorating diseases. No patients showed improvement.
Conclusions: Differential diagnosis of PPFE are various kind of diseases. Only one each patient with metal lung and Sjögren’s syndrome were able to be stabilized.
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