Хронические фиброзирующие интерстициальные заболевания легких с прогрессирующим фиброзным фенотипом: резолюция Междисциплинарного Совета экспертов

2021 
Introduction . The natural course of some interstitial lung diseases (ILD) is characterized by progressive fibrosing phenotype resembling idiopathic pulmonary fibrosis (IPF). Until recently, the antifibrotic drug nintedanib was approved for treatment of the only fibrosing ILD which was IPF. A new indication for this drug which has been registered in Russian Federation in 2021 includes other fibrosing ILDs with progressive phenotype (PF-ILDs) and ILD associated with systemic scleroderma (SS-ILD). The aim of this publication is to describe general considerations of the decision of Multidisciplinary Expert Board on diagnosis and treatment of PF-ILDs including SS-ILD. Results . According to the extension in nintedanib use mentioned above, the Expert Board created an algorithm for diagnosis and treatment of patients with PF-ILDs and criteria for nuntedanib administration in PF-ILDs. Conclusion . Antifibrotic therapy is needed for patients with PF-ILDs with the failure of the stanrard therapy. In those patients antifibrotic treatment should be initiated as early as possible to better preserve the lung function.
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