RT Journal Article
SR Electronic
T1 Idiopathic pulmonary upper lobe fibrosis shows distinct features and poor prognosis compared to idiopathic pulmonary fibrosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P477
VO 42
IS Suppl 57
A1 Makoto Shioya
A1 Mitsuo Otsuka
A1 Yasuo Kitamura
A1 Keiki Yokoo
A1 Kimiyuki Ikeda
A1 Hirotaka Nishikiori
A1 Masanori Shiratori
A1 Hiroki Takahashi
YR 2013
UL http://erj.ersjournals.com/content/42/Suppl_57/P477.abstract
AB Objectives: Idiopathic pulmonary upper lobe fibrosis (IPUF) characterized by its unique distribution of abnormalities occupies a part of idiopathic interstitial pneumonias (IIPs) in spite of being a few. We aimed to clarify the clinical features and prognosis of IPUF in comparison with idiopathic pulmonary fibrosis (IPF).Methods: We subjected 18 IPUF patients (6 male, 12 female) and 49 IPF patients (40 male, 9 female) who were identified on the basis of the medical records of patients with IIPs in our hospital between 2009-‘12. IPUF was diagnosed in consideration of upper lobe dominant distribution of abnormalities regardless having honeycombing on HRCT. Their clinical data including BMI, PFT, serum markers (SP-A, SP-D, KL-6) and TRPG on echocardiography were analyzed. Survival was calculated by the Kaplan-Meier method.Results: Nine of 18 IPUF showed honeycombing. There was no difference in mean age between IPUF and IPF. Rate of female was significantly higher in IPUF. IPUF showed significantly lower rate of smoking habitant (44 v.s. 84%), lower BMI (20.8 v.s. 24.4). IPUF showed high incidence of previous pneumothorax (50%). Significantly lower %FVC (63.1 v.s. 80.0%) was shown in IPUF, while there was no significant difference in %DLco. In IPUF, SP-A was significantly lower (63.3 v.s. 84.2 ng/ml), while SP-D trended to be higher than IPF. IPUF complicated pulmonary hypertension (PH; TRPG > 30 mmHg) more often than IPF (59 v.s. 35%). The survival time was significantly shorter in IPUF.Conclusions: IPUF had different features in many parameters including PFT and serum markers from IPF. Moreover, IPUF had higher incidence of PH and worse outcome than IPF.