ORIGINAL ARTICLEClinical Importance of Bronchoalveolar Lavage Fluid and Blood Cytokines, Surfactant Protein D, and Kerbs von Lungren 6 Antigen in Idiopathic Pulmonary Alveolar Proteinosis
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PARTICIPANTS AND METHODS
The Taipei Veterans General Hospital Institutional Review Board approved this prospective study, and informed consent was obtained from all study participants. Fifteen consecutive patients (10 men [67%], 5 women [33%]) with iPAP diagnosed by characteristic cytopathologic examination of BALF16 and pathologic examination of transbronchial lung biopsy specimens or open lung biopsy specimens were enrolled during a period of 10 years (from January 1, 1995, through June 30, 2005). All patients with
Clinical Data
The demographic data, including sex and smoking habits, showed no substantial difference between patients with iPAP and any subgroup of the diseased controls and lung controls. Patients with iPAP were slightly younger than lung controls (mean ± SD age, 40.6±14.6 vs 52.4±20.4 years; P=.06) and significantly younger than those with ILD (interstitial pneumonitis associated with collagen vascular diseases, 62.6±13.2 years, P<.001; IPF, 68.9±9.2 years, P<.001; sarcoidosis, 53.2±14.4 years, P=.03).
DISCUSSION
To our knowledge, ours is the first single-center study to investigate the clinical importance of BALF and blood levels of SFTPD, KL-6, and various cytokines. Elevated serum and/or BALF levels of SFTPD and KL-6 are reported in patients with iPAP12, 13, 14 and are useful in helping to diagnose iPAP, estimate its disease activity, and monitor for the treatment response of GM-CSF therapy.12, 13, 14, 15
In agreement with previous studies, we found that BALF levels of SFTPD and KL-6 were
CONCLUSION
Lungs of patients with iPAP may be prototypical of chronic inflammation, as evidenced by elevated levels of chemokines and proinflammatory cytokines, SFTPD, and KL-6 in BALF. Furthermore, levels of KL-6 in BALF and blood may serve as a disease severity marker for iPAP and an aid in determining the need for subsequent therapeutic lung lavage.
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Cited by (28)
Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach
2018, The Lancet Respiratory MedicineCitation Excerpt :If there are no obvious exposures to secondary PAP risk factors, the combination of increased GM-CSF concentrations and no measurable anti-GM-CSF antibodies should prompt genetic testing for variant receptor genes. Several other serum analytes have been investigated as surrogate biomarkers of disease severity and progression, but none have become widely accepted, due in part to lack of specificity, limited clinical availability, and longitudinal insensitivity to change (table).52–57 Chest radiographs typically show bilateral alveolar infiltrates.
Pulmonary alveolar proteinosis-like change: A fairly common reaction associated with the severity of idiopathic pulmonary fibrosis
2016, Respiratory InvestigationCitation Excerpt :In such a scenario, although not proven, a PAP-like change can be a reaction indicating dysfunctional clearance in the remodeled lung. Lin et al. and Takahashi et al. reported that serum KL-6 is significantly higher in patients with autoimmune PAP than in patients with IP [22,23]. In the present study, we compared concentrations of KL-6 between patients with IPF with and without a PAP-like change. The KL-6 concentration was significantly higher in patients with a PAP-like change.
Pulmonary alveolar proteinosis: New insights from a single-center cohort of 70 patients
2011, Respiratory MedicineCitation Excerpt :Takahashi et al24 demonstrated that serum and BAL-fluid KL-6 levels were extremely high in 4 patients with PAP. Lin et al30 showed in 15 patients with primary PAP that KL-6 at diagnosis may serve as disease severity marker and as aid in determining the need for subsequent WLL. Our study confirmed the correlation of KL-6 with the magnitude of functional impairment.
Comparison of biomarkers of subclinical lung injury in obstructive sleep apnea
2011, Respiratory MedicineCitation Excerpt :SP-D was recently reported to be a promising lung-specific biomarker in COPD.10 However, results of previous studies suggested that KL-6 was a more discriminative biomarker than SP-D in IPF and collagen vascular disease-associated interstitial pneumonitis,24 idiopathic pulmonary alveolar proteinosis35 or sarcoidosis.36 Madsen et al. reported that SP-D was widely distributed in epithelial cells in a variety of tissues and was not restricted to the respiratory system.37
How We Do It: Whole Lung Lavage
2022, Sarcoidosis Vasculitis and Diffuse Lung Diseases
This work was supported by grants NSC 94-2314-B-075-097 and NSC 95-2314-B-010-026 from the National Science Council of the Republic of China. The study sponsors were not involved in the study design; in the collection, analysis, and interpretation of data; in the writing of the manuscript; or the decision to submit the manuscript for publication.