Abstract
Background: Acute exacerbation (AE) is a potentially fatal form of progression in idiopathic pulmonary fibrosis (IPF) and also reported in other idiopathic interstitial pneumonias (IIPs) than IPF and collagen-vascular disease-associated IP (CVD-IP). As high-resolution computed tomography (HRCT) can provide prognostic information in AE of IPF, we sought to expand the prognostic value of HRCT evaluation at diagnosis from AE of IPF to AE of IIPs and CVD-IP.
Methods: 85 consecutive patients with AE of IIPs or CVD-IP who were hospitalized to Kyoto University Hospital from January 2006 to March 2013 were reviewed retrospectively. Clinical information was collected from medical records, and radiological pattern, disease extent, its asymmetry and diameter ratio of main pulmonary artery/aorta (PA/Ao) on HRCT at diagnosis were assessed. Impacts of clinical and radiological measures on 180-day survival were analyzed.
Results: 180-day survival rate of 85 patients (29 with IPF, 29 other IIPs and 27 CVD-IP) was 51.6%. Univariate analysis revealed that CTD-IP, long-term oxygen therapy, PaO2/FiO2, asymmetrical distribution of consolidation and ground-glass attenuation (GGA) (disease extent more than twice in one lung), PA/Ao, platelet count, lactate dehydrogenase (LDH), Krebs von den Lungen-6 (KL-6) were associated with 180-day mortality. Multivariate analysis revealed that PaO2/FiO2 (hazard ratio 0.99; p=0.01) and asymmetrical distribution (hazard ratio 0.21; p<0.01) were independent predictors of 180-day mortality.
Conclusions: Asymmetrical distribution of opacities on HRCT at diagnosis can predict better prognosis in AE of IP.
- © 2014 ERS