Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan. (August 2018)

• Record Type: Journal Article
• 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
• Is Part Of: Respiratory medicine. Volume 141(2018)
• 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
• DOI: 10.1016/j.rmed.2018.06.022
• Languages: English
• ISSNs: 0954-6111

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 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.