N.G. SHAMSUTDINOVA¹, E.V. DYAKOVA², G.R. SHAKIROVA^1, 2, B.KH. NURULLIN², D.M. YEFIMOVA¹, A.S. VOLKOVA¹, V.A. KURSINA¹

 ¹Kazan State Medical University, Kazan

²Republic Clinical Hospital, Kazan

Contact details:

Shamsutdinova N.G. — PhD (Medicine), Associate Professor of the Department of Hospital Therapy

Address: 49 Butlerov St., Kazan, Russian Federation, 420012, tel.: +7-904-763-83-72, e-mail: ngshamsutdinova@gmail.com

 Interstitial lung diseases (ILD) are a heterogeneous group of diseases characterized by damage to the alveolar walls, interalveolar septa, and pulmonary capillary walls and leading to fibrotic remodeling of these structural components. The processes of fibroblast hyperproliferation and collagen deposition may occur with different severity and speed depending on the pattern prevailing in the ILD structure. Currently, experts identify a progressive fibrotic phenotype — a variant of the ILD course with a rapid increase in pulmonary fibrosis. In the practice of a pulmonologist today, patients with the progressive phenotype remain among the most difficult to manage.

The purpose — to evaluate the effectiveness of antifibrotic therapy in patients observed at the advisory clinic of Republic Clinical Hospital of Tatarstan.

Material and methods. A retrospective analysis of 16 outpatient records of patients diagnosed with ILD with a fibrosing phenotype was conducted before starting an antifibrotic drug (nintedanib) and 6 months after prescription. All patients underwent a general clinical examination, spirometry, a 6-minute walk test, and high-resolution multislice computed tomography at baseline and after 6 months of treatment.

Results. 7 patients (43.8%) were diagnosed with hypersensitivity pneumonitis, 4 with idiopathic pulmonary fibrosis, 4 with ILD-associated autoimmune diseases, 2 with nonspecific interstitial pneumonia, fibrotic phenotype. The median parameters of spirometry and the 6-minute walk test before and after 6 months of therapy are presented.

Conclusion. Antifibrotic therapy is a safe therapy for patients with fibrosing ILD. All patients tolerated nintedanib therapy satisfactorily. The antifibrotic therapy led to stabilization of spirometry parameters.

Key words: interstitial lung diseases, progressive fibrotic phenotype.

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