Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan. (August 2018)
- Record Type:
- Journal Article
- Title:
- Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan. (August 2018)
- Main Title:
- Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan
- Authors:
- Ishii, Hiroshi
Watanabe, Kentaro
Kushima, Hisako
Baba, Tomohisa
Watanabe, Satoshi
Yamada, Yoshihito
Arai, Toru
Tsushima, Kenji
Kondoh, Yasuhiro
Nakamura, Yutaro
Terasaki, Yasuhiro
Hebisawa, Akira
Johkoh, Takeshi
Sakai, Fumikazu
Takemura, Tamiko
Kawabata, Yoshinori
Ogura, Takashi - Abstract:
- Abstract: Background: Pleuroparenchymal fibroelastosis (PPFE) is a rare subset of idiopathic interstitial pneumonias. No large-scale clinical studies of PPFE have been published. The aim of the study was to clarify the clinical and physiological characteristics of PPFE in Japan Methods: This was a retrospective, nationwide, and multicenter study in Japan. We reviewed 52 patients with PPFE, diagnosed after multidisciplinary discussions. Results: Flat chest index, defined as the ratio of anteroposterior diameter to transverse diameter of thoracic cage at the level of 6th thoracic vertebra, correlated positively with body mass index (BMI) ( r = 0.340, p = 0.013) and percentage of predicted value of forced vital capacity (FVC %pred) ( r = 0.355, p = 0.012), and negatively with the ratio of residual volume to total lung capacity (RV/TLC) ( r = −0.312, p = 0.042). RV/TLC correlated negatively with BMI ( r = −0.746, p < 0.0001) and FVC %pred ( r = −0.507, p = 0.0005), and positively with age, and physiological variables (GAP) scores ( r = 0.332, p = 0.030). The median survival time and the cumulative 5-year survival rate were 96 months and 58%, respectively. Patients with KL-6 level >600 (U/mL) survived shorter than those with <600 ( p < 0.001). Conclusion: Low BMI, decreased FVC and increased RV/TLC in PPFE may be related to the progression of flattened chest cage which impairs distension of chest cage at inspiration. Elevated serum levels of KL-6 suggest a poorAbstract: Background: Pleuroparenchymal fibroelastosis (PPFE) is a rare subset of idiopathic interstitial pneumonias. No large-scale clinical studies of PPFE have been published. The aim of the study was to clarify the clinical and physiological characteristics of PPFE in Japan Methods: This was a retrospective, nationwide, and multicenter study in Japan. We reviewed 52 patients with PPFE, diagnosed after multidisciplinary discussions. Results: Flat chest index, defined as the ratio of anteroposterior diameter to transverse diameter of thoracic cage at the level of 6th thoracic vertebra, correlated positively with body mass index (BMI) ( r = 0.340, p = 0.013) and percentage of predicted value of forced vital capacity (FVC %pred) ( r = 0.355, p = 0.012), and negatively with the ratio of residual volume to total lung capacity (RV/TLC) ( r = −0.312, p = 0.042). RV/TLC correlated negatively with BMI ( r = −0.746, p < 0.0001) and FVC %pred ( r = −0.507, p = 0.0005), and positively with age, and physiological variables (GAP) scores ( r = 0.332, p = 0.030). The median survival time and the cumulative 5-year survival rate were 96 months and 58%, respectively. Patients with KL-6 level >600 (U/mL) survived shorter than those with <600 ( p < 0.001). Conclusion: Low BMI, decreased FVC and increased RV/TLC in PPFE may be related to the progression of flattened chest cage which impairs distension of chest cage at inspiration. Elevated serum levels of KL-6 suggest a poor prognosis of PPFE. Highlights: This is the largest case series for PPFE diagnosed after multidisciplinary discussions. Low-compliant thoracic cage, flat chest, modifies the pathophysiology of PPFE. Remarkably decreased FVC and increased RV/TLC are functional characteristics in PPFE. Elevated levels of serum KL-6 suggest a poor prognosis of PPFE. … (more)
- Is Part Of:
- Respiratory medicine. Volume 141(2018)
- Journal:
- Respiratory medicine
- Issue:
- Volume 141(2018)
- Issue Display:
- Volume 141, Issue 2018 (2018)
- Year:
- 2018
- Volume:
- 141
- Issue:
- 2018
- Issue Sort Value:
- 2018-0141-2018-0000
- Page Start:
- 190
- Page End:
- 197
- Publication Date:
- 2018-08
- Subjects:
- Pleuroparenchymal fibroelastosis -- Idiopathic pulmonary fibrosis -- Forced vital capacity -- RV/TLC -- KL-6
BAL bronchoalveolar lavage -- BMI body mass index -- CT computed tomography -- DLco diffusing capacity for carbon monoxide -- FEV1 forced expiratory volume in 1 s -- FVC forced vital capacity -- GAP gender, age and physiological variables -- IIPs idiopathic interstitial pneumonias -- IPF idiopathic pulmonary fibrosis -- KL-6 Krebs von den lungen-6 -- 6MWD 6-mininte walk distance -- 6MWT 6-min walk test -- NSIP nonspecific interstitial pneumonia -- PPFE pleuroparenchymal fibroelastosis -- %pred percentages of predicted values -- RV residual volume -- SpO2 arterial oxygen saturation measured by pulse oximetry -- TLC total lung capacity -- UIP usual interstitial pneumonia
Chest -- Diseases -- Periodicals
Chest -- Diseases -- Great Britain -- Periodicals
Respiratory organs -- Diseases -- Periodicals
Respiratory Tract Diseases -- Periodicals
Appareil respiratoire -- Maladies -- Périodiques
Thorax -- Maladies -- Périodiques
Appareil respiratoire -- Maladies -- Traitement -- Périodiques
Electronic journals
616.2 - Journal URLs:
- http://www.sciencedirect.com/science/journal/09546111 ↗
http://www.clinicalkey.com/dura/browse/journalIssue/09546111 ↗
http://www.clinicalkey.com.au/dura/browse/journalIssue/09546111 ↗
http://www.elsevier.com/journals ↗ - DOI:
- 10.1016/j.rmed.2018.06.022 ↗
- Languages:
- English
- ISSNs:
- 0954-6111
- Deposit Type:
- Legaldeposit
- View Content:
- Available online (eLD content is only available in our Reading Rooms) ↗
- Physical Locations:
- British Library DSC - 7777.661900
British Library DSC - BLDSS-3PM
British Library STI - ELD Digital store - Ingest File:
- 12822.xml