Abstract
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.
- © 2013 ERS
