Hiroshi Ishii1, Kentaro Watanabe2, Hisako Kushima1, Tomohisa Baba3, Satoshi Watanabe4, Yoshihito Yamada5, Toru Arai6, Kenji Tsushima7, Yasuhiro Kondoh8, Yutaro Nakamura9, Yasuhiro Terasaki10, Akira Hebisawa11, Takeshi Johkoh12, Fumikazu Sakai13, Tamiko Takemura14, Yoshinori Kawabata15, Takashi Ogura3. 1. Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan. 2. Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan. Electronic address: watanabe@fukuoka-u.ac.jp. 3. Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Kanagawa, Japan. 4. Department of Respiratory Medicine, Cellular Translation Biology, Kanazawa University Graduate School of Medicine, Japan. 5. Department of Respiratory Medicine, JR Tokyo General Hospital, Japan. 6. Department of Respiratory Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Japan. 7. Department of Respiratory Medicine, Chiba University Hospital, Japan. 8. Department of Respiratory Medicine, Tosei General Hospital, Japan. 9. Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Japan. 10. Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan. 11. Department of Clinical Research, National Hospital Organization Tokyo National Hospital, Japan. 12. Department of Radiology, Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Hyogo, Japan. 13. Department of Diagnostic Radiology, Saitama International Medical Center, Saitama Medical University, Saitama, Japan. 14. Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan. 15. Division of Pathology, Saitama Cardiovascular and Respiratory Center, Saitama, Japan.
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 poor prognosis of PPFE.
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.
Authors: Felix Chua; Sujal R Desai; Andrew G Nicholson; Anand Devaraj; Elisabetta Renzoni; Alexandra Rice; Athol U Wells Journal: Ann Am Thorac Soc Date: 2019-11