Heterogeneous clinical features in patients with pulmonary fibrosis showing histology of pleuroparenchymal fibroelastosis.
BACKGROUND
The histological pattern of pleuroparenchymal fibroelastosis (PPFE) is well defined, but its clinical features remain unclear.


METHODS
We retrospectively examined the predominantly involved lung-fields (based on abnormal opacities on computed tomography [CT] images), and the initial value and annual decline of respiratory function in patients with pulmonary fibrosis presenting with histologically confirmed PPFE.


RESULTS
Thirteen female and nine male subjects were included. Eleven interpreters independently analyzed 231 CT image series. One-third of the CT series (78/231) was interpreted as demonstrating equal involvement of the upper and lower lung fields, i.e., six out of 21 patients had equal involvement of the upper and lower lung fields, based on a majority decision of the interpreters. The residual volume/total lung capacity (RV/TLC) was increased and correlated inversely with forced vital capacity (FVC) at the initial measurement. FVC followed two patterns of decline over time: a gradual decline over a follow-up period of more than 6 years (-55mL/year, R(2)=0.799), and a relatively rapid decline over a shorter period (-364mL/year, R(2)=0.855) as determined by mixed-effect linear regression.


CONCLUSIONS
The predominantly involved sites seen on CT images of PPFE were not limited to the upper lobes. In some cases, upper lung fields were predominantly involved, but in other cases, both upper and lower lung fields were equally involved. Two patterns of FVC decline exists: a rapid decline over a short period and a slow decline over a longer period, suggesting that the disease follows a heterogeneous clinical course.
Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: sukehiko_yossan@yahoo.co.jp.
Yuji
Y
Yoshida
Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka 818-0067, Japan. Electronic address: nbnagata@fukuoka-u.ac.jp.
Nobuhiko
N
Nagata
Department of Respiratory Medicine, Hamanomachi Hospital, Fukuoka 810-8539, Japan. Electronic address: tsuruta@hamanomachi.jp.
Nobuko
N
Tsuruta
Department of Respiratory Medicine, Kurume General Hospital, Kurume 830-0013, Japan. Electronic address: kita-sir1028@jcom.home.ne.jp.
Yasuhiko
Y
Kitasato
Department of Respiratory Medicine, National Hospital Organization, Omuta National Hospital, Omuta 837-0911, Japan. Electronic address: wakamatsu-k@oomuta-h.com.
Kentaro
K
Wakamatsu
Department of Respiratory Medicine, National Hospital Organization, Fukuoka Higashi Medical Center, Koga 811-3195, Japan. Electronic address: yoshimimi@fukuokae2.hosp.go.jp.
Michihiro
M
Yoshimi
Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: hishii@fukuoka-u.ac.jp.
Hiroshi
H
Ishii
Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: tahirota@fukuoka-u.ac.jp.
Takako
T
Hirota
Department of Respiratory Medicine, Kyushu University Hospital, Fukuoka 812-8582, Japan. Electronic address: n-hamada@kokyu.med.kyushu-u.ac.jp.
Naoki
N
Hamada
Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: mfujita@fukuoka-u.ac.jp.
Masaki
M
Fujita
Department of Pathology, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: kaznabes@fukuoka-u.ac.jp.
Kazuki
K
Nabeshima
Academia, Industry, and Government Collaborative Research Institute of Translational Medicine for Life Innovation, Fukuoka University, Fukuoka 814-0180, Japan. Electronic address: fumkiyomi@adm.fukuoka-u.ac.jp.
Fumiaki
F
Kiyomi
Department of Respiratory Medicine, Fukuoka University School of Medicine, Fukuoka 814-0180, Japan. Electronic address: watanabe@fukuoka-u.ac.jp.
Kentaro
K
Watanabe
D000368:Aged; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011658:Pulmonary Fibrosis / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed
Forced vital capacity (FVC); Idiopathic interstitial pneumonia (IIP); Idiopathic pulmonary fibrosis (IPF); Pleuroparenchymal fibroelastosis (PPFE); Pulmonary upper lobe fibrosis