RT Journal Article
SR Electronic
T1 Clinical investigation of idiopathic pleuroparenchymal fibroelastosis cases diagnosed by radiologic findings and fibroelastosis obtained by TBLB
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 3218
VO 44
IS Suppl 58
A1 Ryota Sato
A1 Shinobu Akagawa
A1 Nobuharu Ohshima
A1 Keita Takeda
A1 Takahiro Ando
A1 Kazuya Koyama
A1 Ken-ichi Okuda
A1 Masahiro Shimada
A1 Masahiro Kawashima
A1 Kimihiko Masuda
A1 Hirotoshi Matsui
A1 Akira Hebisawa
A1 Ken Ohta
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/3218.abstract
AB Background:Idiopathic pleuroparenchymal fibroelastosis (IPPFE) was newly included in ATS/ERS classification as a rare IIP, and fibroelastosis (FE) is one of the fundamental histological findings. Because performing VATS is often difficult, the clinical diagnosis remained unconfirmed.Objectives:We aimed to clarify the clinical features of IPPFE cases which were diagnosed by radiologic findings and histological FE in upper lobes obtained by TBLB.Methods:We selected cases with FE proven by TBLB in our hospital from 2003 to 2012. PPFE was diagnosed based on pleuroparenchymal thickening and volume loss of upper lobes as well as upper lobe dominant radiological abnormalities.Results:Among 27 cases with FE detected by TBLB, clinical diagnoses suspected before TBLB were PPFE in 11 cases, pulmonary aspergillosis in 6, non-tuberculous mycobacteriosis (NTM) in 3, and others in 7. Finally, 14 cases were diagnosed as PPFE, including two cases of suspected association with collagen vascular diseases. In 12 IPPFE cases (9 male, 3 female, average age 66.8), 6 had thinness (BMI<18.5). Clues to the diagnosis were abnormalities in chest radiographs in 5 cases, and cough in 5. Seven cases had obstructive disorder and 5 had pneumothorax/pneumomediastinum in their clinical course. Aspergillosis was accompanied in 4 cases, and NTM in 2. Eight of 10 followed-up cases gradually deteriorated.Conclusions: Clinical features of IPPFE cases diagnosed by radiologic findings and histological FE by TBLB are similar to those of IPPFE cases diagnosed by VATS reported previously. Comprehensive approach including radiograph is important to diagnose IPPFE.