Abstract
Background and objective: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar filling. YKL-40, a chitinase-like protein produced by macrophages and epithelial cells, is increased in patients with interstitial lung diseases. We aimed to evaluate the role of YKL-40 as a biomarker for PAP. Methods: A total of 34 patients with autoimmune PAP and 50 healthy controls were studied. YKL-40 was measured by ELISA in serum and bronchoalveolar lavage fluid (BALF). Chitinase coding gene polymorphisms (CHI3L1-329 and -131) were detected by PCR and pyrosequencing. Correlations between serum YKL-40 levels and disease outcome were analysed. Results: Baseline serum and BALF levels of YKL-40 were higher in PAP patients than in controls (286 +/- 27 ng/mL vs 42 +/- 4 ng/mL, P < 0.0001;323 +/- 36 ng/mL vs 3 +/- 1 ng/mL, P < 0.0001, respectively). Serum YKL-40 levels correlated with diffusing capacity of the lung for carbon monoxide (DLCO) at baseline (P = 0.002) and over time (P < 0.0001). Patients with disease progression had higher baseline serum YKL-40 levels than those who remained stable or improved (P < 0.0001). A baseline cut-off level of 300 ng/mL was predictive of disease progression (HR (hazard ratio): 7.875, P = 0.001). The presence of the G allele was associated with higher serum and BALF levels of YKL-40. Conclusion: YKL-40 is elevated in serum and BALF of PAP patients, and may be of clinical utility to predict outcome in PAP.
Dokumententyp: | Zeitschriftenartikel |
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Fakultät: | Medizin |
Themengebiete: | 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit |
ISSN: | 1323-7799 |
Sprache: | Englisch |
Dokumenten ID: | 50274 |
Datum der Veröffentlichung auf Open Access LMU: | 14. Jun. 2018, 09:43 |
Letzte Änderungen: | 04. Nov. 2020, 13:27 |