PT  - JOURNAL ARTICLE
AU  - Ryota Sato
AU  - Shinobu Akagawa
AU  - Nobuharu Ohshima
AU  - Keita Takeda
AU  - Takahiro Ando
AU  - Kazuya Koyama
AU  - Ken-ichi Okuda
AU  - Masahiro Shimada
AU  - Masahiro Kawashima
AU  - Kimihiko Masuda
AU  - Hirotoshi Matsui
AU  - Akira Hebisawa
AU  - Ken Ohta
TI  - Clinical investigation of idiopathic pleuroparenchymal fibroelastosis cases diagnosed by radiologic findings and fibroelastosis obtained by TBLB
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - 3218
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/3218.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/3218.full
SO  - Eur Respir J2014 Sep 01; 44
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.