Abstract
BackgroundWe hypothesized that, in patients with pulmonary fibrosis combined with emphysema, clinical characteristics and outcomes may differ from patients with pulmonary fibrosis without emphysema. We identified 102 patients who met established criteria for pulmonary fibrosis. The amount of emphysema (numerical score) and type of emphysema (centrilobular, paraseptal, or mixed) were characterized in each patient. Clinical characteristics, pulmonary function tests and patient survival were analysed.ResultsBased on the numerical emphysema score, patients were classified into those having no emphysema (n = 48), trivial emphysema (n = 26) or advanced emphysema (n = 28). Patients with advanced emphysema had a significantly higher amount of smoking in pack/years than patients with no emphysema or trivial emphysema (P < 0.0001). Median survival [1st, 3rd quartiles] of patients with advanced emphysema was 63 [36, 82] months compared to 29 [18, 49] months in patients without emphysema and 32 [19, 48] months in patients with trivial emphysema (P < 0.001). Median forced vital capacity (FVC) and total lung capacity (TLC) were higher in the advanced emphysema group compared to patients with no emphysema (P < 0.01 and P < 0.001, respectively), whereas median DLCO did not differ among groups and was overall low. Within the advanced emphysema group (n = 28), further characterization of the type of emphysema was performed and, within these subgroups of patients, survival was 75 [58, 85] months for patients with centrilobular emphysema, 75 [48, 85] months for patients with mixed centrilobular/paraseptal emphysema, and 24 [22, 35] months for patients with paraseptal emphysema (P < 0.01). Patients with advanced paraseptal emphysema had similar survival times to patients without emphysema.ConclusionsPatients with pulmonary fibrosis combined with advanced centrilobular or mixed emphysema have an improved survival compared with patients with pulmonary fibrosis without emphysema, with trivial emphysema or with advanced paraseptal emphysema.
Highlights
We hypothesized that, in patients with pulmonary fibrosis combined with emphysema, clinical characteristics and outcomes may differ from patients with pulmonary fibrosis without emphysema
Patients with connective tissue diseases such as systemic sclerosis develop pulmonary fibrosis but this occurs in association with autoimmune inflammation and the survival of patients with interstitial pneumonia associated with connective tissue diseases is longer than patients with idiopathic pulmonary fibrosis (IPF) [6,7]
We addressed the hypothesis that the prognosis of patients with pulmonary fibrosis combined with emphysema may be different from patients with pulmonary fibrosis in the absence of emphysema
Summary
In patients with pulmonary fibrosis combined with emphysema, clinical characteristics and outcomes may differ from patients with pulmonary fibrosis without emphysema. Pulmonary fibrosis is a major component of various diffuse parenchymal lung diseases, including idiopathic pulmonary fibrosis (IPF) and other forms of idiopathic interstitial pneumonia. We previously reported that lymphocytic inflammation induced by gene delivery of chemokine CCL18 is partially protective against the severe fibrosis caused by administration of bleomycin in mice [8]. Based on these considerations, we hypothesized that patients with IPF combined with a chronic inflammatory process may be partially protected from the usual rapidly declining clinical course. Emphysema is most commonly caused by cigarette smoking [9] and the inflammatory component of emphysema may be only partially reversed after smoking cessation [11]
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