Clinical Impact of Emphysema Evaluated by High-Resolution Computed Tomography on Idiopathic Pulmonary Fibrosis Diagnosed by Surgical Lung Biopsy

Abstract

Background: The prognosis of combined cases of pulmonary fibrosis and emphysema is unresolved partially because radiological differentiation between usual interstitial pneumonia and nonspecific interstitial pneumonia is difficult in coexisting emphysema cases. Objective: The purpose of this study was to clarify the clinical impact of emphysema on the survival of patients with idiopathic pulmonary fibrosis (IPF). Methods: One hundred and seven patients with interstitial lung diseases were diagnosed by surgical lung biopsies between 2006 and 2012, and 47 patients were diagnosed with IPF through multidisciplinary discussion. Emphysema on high-resolution computed tomography scans was evaluated semiquantitatively by visual scoring. Results: Eight out of the 47 IPF patients showed a higher emphysema score (>3) and were diagnosed to have IPF-emphysema. The median survival time of patients with IPF-emphysema (1,734 days) from the initial diagnosis was significantly shorter than that of patients with IPF alone (2,229 days) by Kaplan-Meier analysis (p = 0.007, log-rank test). Univariate Cox proportional hazard regression analyses revealed that a higher total emphysema score (>3.0) was a significantly poor prognostic factor in addition to Krebs von den Lungen-6, surfactant protein-D, arterial oxygen tension, percent forced vital capacity, and percent diffusing capacity of carbon monoxide (%D<smlcap>LCO</smlcap>). Multivariate Cox proportional hazard regression analyses with the stepwise method showed that higher total emphysema score (>3) and %D<smlcap>LCO</smlcap> were significantly poor prognostic factors. Conclusions: The prognosis of IPF-emphysema was significantly worse than that of IPF alone.

Comorbid Conditions in Idiopathic Pulmonary Fibrosis: Recognition and Management

“…The recognition of CPFE has potential management implications. Some studies suggest that CPFE is associated with reduced survival ( 21 , 23 , 25 ), but others have not replicated this observation ( 22 , 26 ). Paradoxically, patients with CPFE appear to have a slower rate of FVC decline, perhaps due to the impact of emphysema on the manner in which FVC reflects progressive fibrosis ( 27 ).…”

Idiopathic Pulmonary Fibrosis in Switzerland: Diagnosis and Treatment

“…It is unclear whether CPFE is a single disease or the simultaneous occurrence of 2 disorders sharing 1 or more risk factors such as smoking. Emphysema in IPF patients is associated with worse prognosis [72] . The fibrotic component of CPFE could correspond to UIP on imaging or histologically.…”

Comorbidities and Complications in Idiopathic Pulmonary Fibrosis

“…Some studies suggest that CPFE is associated with reduced survival [ 7 ], but others have not replicated this observation [ 2 , 7 ]. Paradoxically, patients with CPFE have a slow rate of FVC decline, perhaps due to the impact of emphysema on the manner in which FVC reflects progressive fibrosis [ 8 ]. An increased prevalence of pulmonary hypertension (PH) has also been demonstrated among those with CPFE [ 2 , 7 ], which may also impact survival [ 2 , 8 ] and is the principal negative prognostic factor for this condition.…”

“…However, the whole spectrum of severity of emphysema was not represented in those studies, because those patients who presented an obstructive ventilatory pattern or a very severe reduction of DLCO on lung function testing were excluded. It is unclear whether patients with IPF and CPFE can benefit from treatments with inhaled drugs, such as long-acting beta-agonists, long-acting muscarinic antagonists, and inhaled corticosteroids [ 2 , 6 , 7 , 8 ]. We think that clinicians should consider the addition of these therapies, according to chronic obstructive pulmonary disease consensus guidelines [ 8 ], but this is an empiric non-evidence-based recommendation.…”