Systemic scleroderma is a systemic autoimmune disease that affects multiple organs, including the bronchopulmonary system. The disease leads to progressive pulmonary fibrosis, which is the most common cause of death. Hence, standard therapy of systemic sclerosis should be supplemented with antifibrotic therapy. The article describes a clinical case of the efficacy of a combination of standard therapy with the tyrosine kinase inhibitor nintedanib in a patient with systemic scleroderma and a progressive phenotype ILD. The patient was taking nintedanib in combination with standard therapy (methylprednisolone, mycophenolate mofetil) for 7 months and showed improvement on CT in the form of a significant decrease in the ground glass lesions. In addition, bodyplethysmography showed a decrease in restrictive changes in the external respiration (an increase in the total lung capacity from 49 to 57%), an improvement in the lung diffusion capacity (an increase in total diffusion from 47 to 53%). This clinical case demonstrates the high efficacy of antifibrotic drug nintedanib in the complex therapy of patients with systemic scleroderma and progressive pulmonary fibrosis. Nintedanib increases the efficacy of basic therapy and improves the prognosis.

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