ABSTRACT
Combined pulmonary fibrosis and emphysema (CPFE) is an under-recognized syndrome for which the diagnostic use of serum biomarkers is an attractive possibility. We hypothesized that CC16 and/or TGF-ß1 or combinations with other biomarkers are useful for diagnosing CPFE. Patients with respiratory symptoms and a smoking history, with or without chronic obstructive pulmonary disease, were divided into the following three groups according to findings of high-resolution computed tomography of the chest: controls without either emphysema or fibrosis, patients with emphysema alone, and patients compatible with the diagnosis of CPFE. Serum concentrations of CC16, TGF-ß1, SP-D, and KL-6 were measured in patients whose condition was stable for at least 3 months. To investigate changes in biomarkers of lung fibrosis in patients with a life-long smoking history, additional measurements were performed on the patients with idiopathic pulmonary fibrosis (IPF) of smoking history. The mean age of the first three groups was 68.0 years, whereas that of the IPF group was 71.8 years, and the groups contained 36, 115, 27, and 10 individuals, respectively. The serum concentration of CC16 in the four groups was 5.67 ± 0.42, 5.66 ± 0.35, 9.38 ± 1.04 and 22.15 ± 4.64 ng/ml, respectively, indicating that those patients with lung fibrosis had a significantly higher concentration. The combined use of CC16, SP-D, and KL-6 provided supportive diagnosis in conjunction with radiological imaging in diagnosis of CPFE. We conclude that a combination of biomarkers including CC16 could provide useful information to screen and predict the possible diagnosis of CPFE.
Subject(s)
Pulmonary Emphysema/diagnosis , Pulmonary Fibrosis/diagnosis , Uteroglobin/blood , Aged , Aged, 80 and over , Biomarkers/blood , Case-Control Studies , Female , Humans , Male , Middle Aged , Mucin-1/blood , Pulmonary Emphysema/blood , Pulmonary Fibrosis/blood , Pulmonary Surfactant-Associated Protein D/blood , ROC Curve , Syndrome , Tomography, X-Ray Computed , Transforming Growth Factor beta1/bloodABSTRACT
OBJECTIVE: This study was aimed to investigate the effect of polymyxin B-immobilized fiber column (PMX) hemoperfusion treatment on the acute exacerbation of idiopathic pulmonary fibrosis (IPF). PATIENTS AND METHODS: Six patients with a clinical diagnosis of idiopathic pulmonary fibrosis (IPF) who developed acute exacerbation were included in this study. Although five of six patients were treated with high-dose corticosteroid therapy, mechanical ventilation was necessary for all six patients due to severe respiratory failure. Blood endotoxin levels were undetectable in all patients. PMX treatment was performed on these six patients. RESULTS: In four of six patients, alveolar-arterial difference of oxygen (AaDO(2)), serum KL-6 and lactate dehydrogenase (LDH) were improved after PMX treatment. These four patients were successfully weaned from mechanical ventilation and survived more than 30 days after the initial PMX treatment. CONCLUSION: These data suggest a potential beneficial effect of PMX treatment on acute exacerbation of IPF.
