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Background:Progressive fibrosing interstitial lung diseases have a poor prognosis and may be resistant to corticosteroids and/or immunosuppressants, but antifibrotic therapies such as nintedanib and pirfenidone have been shown to slow the deterioration of lung function. The aim of this study is to identify the characteristic cellular profile of bronchoalveolar lavage fluid at diagnosis for predicting progressive fibrosing interstitial lung diseases.Methods:Patients with interstitial lung diseases were divided into fibrotic and non-fibrotic phenotypes by chest high-resolution computed tomography. The progressive fibrosing phenotype was determined to be fibrotic diseases with more than a 5% relative decline in the percent predicted value of forced vital capacity over 6 months. The proportions of T cell, B cell, monocyte, and macrophage subsets and the degree of T cell activation in peripheral blood and bronchoalveolar lavage fluid were measured by flowcytometry.Results:Fibrotic interstitial lung diseases (n=27) have a Th2-dominant phenotype, whereas non-fibrotic diseases (n=13) have a Th1/Th17-dominant phenotype in bronchoalveolar lavage fluid. In patients with fibrotic diseases, the proportion of Th2 cells, but not the serum KL-6 level or the proportion of lymphocytes, was significantly higher in patients with progressive fibrosing phenotype than in those with non-progressive phenotype, and the receiver operating characteristic curve analysis showed an AUC of 0.79. Thus, a greater population of Th2 cells in bronchoalveolar lavage fluid was diagnostic for progressive fibrosing phenotype.Conclusions:An increased proportion of Th2 cells in bronchoalveolar lavage fluid is associated with greater deterioration of lung function in fibrosing interstitial lung diseases.Trial registration:This study was was registered in the UMIN Clinical Trials Registry (Registry ID UMIN000041871) on October 1, 2020. |
