Pleuroparenchymal fibroelastosis diagnosed by multidisciplinary discussions in Japan - 31/07/18
, Hisako Kushima a, Tomohisa Baba b, Satoshi Watanabe c, Yoshihito Yamada d, Toru Arai e, Kenji Tsushima f, Yasuhiro Kondoh g, Yutaro Nakamura h, Yasuhiro Terasaki i, Akira Hebisawa j, Takeshi Johkoh k, Fumikazu Sakai l, Tamiko Takemura m, Yoshinori Kawabata n, Takashi Ogura bthe Tokyo Diffuse Lung Disease Study Group
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.
Le texte complet de cet article est disponible en PDF.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. |
Keywords : Pleuroparenchymal fibroelastosis, Idiopathic pulmonary fibrosis, Forced vital capacity, RV/TLC, KL-6
Abbreviations : BAL, BMI, CT, DLco, FEV1, FVC, GAP, IIPs, IPF, KL-6, 6MWD, 6MWT, NSIP, PPFE, %pred, RV, SpO2, TLC, UIP
Plan
Vol 141
P. 190-197 - août 2018 Retour au numéro
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