Extent of pulmonary fibrosis on high-resolution computed tomography is a prognostic factor in patients with pleuroparenchymal fibroelastosis |
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| Affiliation: | 1. Department of Clinical Oncology, Hiroshima University Hospital, Hiroshima, Japan;2. Department of Respiratory Internal Medicine, Hiroshima University Hospital, Hiroshima, Japan;3. Department of Molecular and Internal Medicine, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan;4. Department of Diagnostic Radiology, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan;5. Department of Emergency and Critical Care Medicine, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan;6. Department of Physical Analysis and Therapeutic Sciences, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan;1. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan;2. Department of Medical IT Center, Nagoya University Hospital, Japan;3. Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Japan;4. Division of Clinical Epidemiology, Graduate School of Public Health, St. Luke''s International University, Tokyo, Japan;5. Department of Respiratory Medicine and Allergy, Sapporo Medical University School of Medicine, Sapporo, Japan;1. Department of Respiratory Medicine, Cellular Transplantation Biology, Kanazawa University Graduate School of Medicine, 13-1, Takara-machi, Kanazawa, Ishikawa 920-8641, Japan;2. Department of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, Waehringer Guertel 18-20, Vienna 1090 Austria;3. Department of Respiratory Medicine, Komatsu Municipal Hospital, Ho-60, Mukaimoto-ori-machi, Komatsu 923-8560, Japan;4. Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, 3-1 Kurumazuka, Itami, Hyogo 664-8533, Japan;5. Department of Radiology, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga 849-8501, Japan;6. Division of Diagnostic Pathology, Saitama Prefectural Cardiovascular and Respiratory Center, 1696 Itai, Kumagaya City, Saitama 360-0105, Japan;7. Section of Diagnostic Pathology, Kanazawa University Graduate School of Medicine, 13-1, Takara-machi, Kanazawa, Ishikawa 920-8641, Japan;8. Department of Respiratory Medicine, Kanazawa Municipal Hospital, 3-7-3, Heiwa-machi, Kanazawa, Ishikawa 921-8105, Japan;9. Department of Respiratory Medicine, National Hospital Organization Nanao Hospital, 3-1, Mattoumachi-hachibu, Nanao, Ishikawa, Japan;1. General Medical Research Center, Fukuoka University School of Medicine, 814-0180 Fukuoka, Japan;2. Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan;3. Clinical Research Support Center Kyushu, Fukuoka, Japan;4. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan;5. Department of Clinical Research, National Hospital Organization Tokyo National Hospital, Japan;6. Division of Diagnostic Pathology, Saitama Cardiovascular and Respiratory Center, Saitama, Japan;7. Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Hyogo, Japan;8. Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, Saitama, Japan;9. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Aichi, Japan;10. Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Osaka, Japan;11. Department of Pulmonary Medicine, Nippon Medical School, Tokyo, Japan;12. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan;13. Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Kanagawa, Japan;14. Department of Respiratory Medicine, Tokyo Medical and Dental University, Japan;15. Department of Respiratory Medicine, Toho University Omori Medical Center, Japan |
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| Abstract: | ![]()
BackgroundSeveral prognostic factors for pleuroparenchymal fibroelastosis (PPFE) have recently been reported. However, detailed high-resolution computed tomography (HRCT) findings have not yet been evaluated as prognostic factors. This study retrospectively investigated whether HRCT findings are prognostic factors in patients with PPFE compared to those with idiopathic pulmonary fibrosis (IPF).MethodsPatients with PPFE and IPF diagnosed at our hospital between January 2008 and December 2016 were enrolled. Clinical and HRCT characteristics were obtained. In addition to our patients, we also analyzed data of PPFE patients whose cause of death had been identified in previous studies.ResultsWe enrolled 15 patients with PPFE and 75 patients with IPF. Consolidation and maximum pleural thickening were significantly higher in patients with PPFE than in those with IPF (both P < .001). Fibrosis score, honeycomb area, and traction bronchiectasis were not significantly different between these patient groups but were significant prognostic factors in patients with PPFE in univariate analysis (P = .021, P = .017, and P = .014, respectively). The proportions of deaths by acute exacerbation or lung cancer were significantly lower in patients with PPFE than in those with IPF (P < .001 and P = .001, respectively), whereas death by respiratory failure was significantly more frequent in PPFE patients (P < .001).ConclusionsHRCT findings, such as fibrosis score, honeycomb area, and traction bronchiectasis, were independent prognostic factors in patients with PPFE. Respiratory failure, but not acute exacerbation and lung cancer, was the main cause of death in patients with PPFE. |
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| Keywords: | Pleuroparenchymal fibroelastosis High-resolution computed tomography Pulmonary fibrosis Honeycomb pattern AE" },{" #name" :" keyword" ," $" :{" id" :" kwrd0035" }," $$" :[{" #name" :" text" ," _" :" acute exacerbation APDT" },{" #name" :" keyword" ," $" :{" id" :" kwrd0045" }," $$" :[{" #name" :" text" ," _" :" anteroposterior diameter of the thoracic cage BMI" },{" #name" :" keyword" ," $" :{" id" :" kwrd0055" }," $$" :[{" #name" :" text" ," _" :" body mass index CI" },{" #name" :" keyword" ," $" :{" id" :" kwrd0065" }," $$" :[{" #name" :" text" ," _" :" confidence interval CT" },{" #name" :" keyword" ," $" :{" id" :" kwrd0075" }," $$" :[{" #name" :" text" ," _" :" computed tomography diffusing capacity of the lung for carbon monoxide forced expiratory volume in 1.0 s FVC" },{" #name" :" keyword" ," $" :{" id" :" kwrd0105" }," $$" :[{" #name" :" text" ," _" :" forced vital capacity GAP" },{" #name" :" keyword" ," $" :{" id" :" kwrd0115" }," $$" :[{" #name" :" text" ," _" :" gender age" },{" #name" :" keyword" ," $" :{" id" :" kwrd0125" }," $$" :[{" #name" :" text" ," _" :" and physiology GGA" },{" #name" :" keyword" ," $" :{" id" :" kwrd0135" }," $$" :[{" #name" :" text" ," _" :" ground-glass attenuation HR" },{" #name" :" keyword" ," $" :{" id" :" kwrd0145" }," $$" :[{" #name" :" text" ," _" :" hazard ratio HRCT" },{" #name" :" keyword" ," $" :{" id" :" kwrd0155" }," $$" :[{" #name" :" text" ," _" :" high-resolution computed tomography IPF" },{" #name" :" keyword" ," $" :{" id" :" kwrd0165" }," $$" :[{" #name" :" text" ," _" :" idiopathic pulmonary fibrosis KL-6" },{" #name" :" keyword" ," $" :{" id" :" kwrd0175" }," $$" :[{" #name" :" text" ," _" :" Krebs von den Lungen-6 NSIP" },{" #name" :" keyword" ," $" :{" id" :" kwrd0185" }," $$" :[{" #name" :" text" ," _" :" non-specific interstitial pneumonia PPFE" },{" #name" :" keyword" ," $" :{" id" :" kwrd0195" }," $$" :[{" #name" :" text" ," _" :" pleuroparenchymal fibroelastosis TDT" },{" #name" :" keyword" ," $" :{" id" :" kwrd0205" }," $$" :[{" #name" :" text" ," _" :" transverse diameter of the thoracic cage UIP" },{" #name" :" keyword" ," $" :{" id" :" kwrd0215" }," $$" :[{" #name" :" text" ," _" :" usual interstitial pneumonia |
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