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Bonella, Francesco; Ohshimo, Shinichiro; Miaotian, Cai; Griese, Matthias; Guzman, Josune und Costabel, Ulrich: Serum KL-6 is a predictor of outcome in pulmonary alveolar proteinosis. In: Orphanet Journal of Rare Diseases 2013, 8:53


Background: Pulmonary alveolar proteinosis (PAP) is a rare disorder characterised by abundant alveolar accumulation of surfactant lipoproteins. Serum levels of KL-6, high molecular weight human MUC1 mucin, are increased in the majority of patients with PAP. The prognostic significance of KL-6 in PAP is still unknown. Aim of the study was to evaluate whether serum KL-6 levels correlate with the outcome of the disease. Patients and methods: From 2006 to 2012, we prospectively studied 33 patients with primary autoimmune PAP. We measured serum KL-6 levels by ELISA (Eisai, Tokyo, Japan), and evaluated the correlation between initial KL-6 levels and clinical variables. Disease progression was defined as deterioration of symptoms, and/or lung function, and/or chest imaging. Main results: The initial serum KL-6 levels were significantly correlated with the baseline PaO2, A-aDO(2), DLCO, VC and TLC (p=0.042, 0.012, 0.012, 0.02 and 0.013, respectively). The change over time of serum KL-6 correlated with the change over time of DLCO (p=0.017). The initial serum KL-6 levels were significantly higher in patients with disease progression than in those with remission (p<0.001). At a cut-off level of 1526 U/mL, the initial serum KL-6 level predicted disease progression (Se 81\%, Sp 94\%). At a cut-off level of 2157 U/mL, the initial serum KL-6 predicted the necessity of repeated whole lung lavage (Se 83\%, Sp 96\%). In the multivariate analysis, the initial serum level of KL-6 was the strongest predictor of disease progression (HR 9.41, p=0.008). Conclusions: Serum KL-6 seems to predict outcome in PAP.