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• W4387247839 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2023 12:00 pm - 12:45 pm PURPOSE: Systemic sclerosis (SSc) is systemic autoimmune disease, characterized by immune dysregulation, microvascular damage, and inflammation, resulting in progressive fibrosis of the skin and internal organs. Interstitial lung disease (ILD) is a common manifestation of SSc, affecting more than 60% of patients. Despite current immunosuppressants, SSc-ILD may progress, so there is a need for new therapies targeting both inflammation and fibrosis, able to modify this poor prognosis. One of these drugs is Nintedanib. However, there are currently a limited number of studies of this drug in patients with SSc-ILD. PURPOSE: To study the effect of Nintedanib on the main clinical manifestations (pulmonary function, cutaneous fibrosis, signs of cardiopathy), disease activity and quality of life in patients with SSc-ILD. METHODS: 20 patients (the average age 52.7±11.9 years, females 90%, SSc duration 10±6.3 years, diffused:limited forms 1:3.5) with the confirmed SSc diagnosis and signs of progressive ILD were enrolled into the study. All patients received low- and moderate-dose of prednisolone regimens. Nintedanib was added to immunosuppressants (Mycophenolate mofetil, Azathioprine, Cyclophosphamide) due to inadequate efficacy of the previous therapy. 13 patients received Nintedanib (300 mg per day) over the follow-up period 9,7±6,2 months. The time courses FVC(%), DLCO (%), 6-MWT (meters), dyspnea index (NYHA), modified skin count (mRss, points), activity index (EScSG, points), left ventricle ejection fraction (LVEF,%), pulmonary arterial systolic pressure (EchoCG), and cardiac rhythm and conductivity disorders (ECG), diastolic dysfunction, HAQ, S-HAQ, ESR, CRP were evaluated. RESULTS: The therapy was associated with significant decrease in EScSG (from 3.96±1.6 to 1.7±1.1, р=0.0015), increase in tolerance for exercise (for a 6-MWT, from 398,3±108,5m to447±105,5m, p=0,047) and stabilization of FVC and DLCO indicators. A decrease in the dyspnea index (from III to II according to NYHA) was observed in 8 (62%) pts, a downward trend in PASP - in 5 (38%) patients, 5% and more FVC increase -in the third of the pts (4/30%). During the follow-up period no change of mRss, LVEF,%, cardiac rhythm disorders (ECG), in the quality of life and indicators of laboratory inflammatory activity was observed. Against the background of therapy, there was a tendency to increase the number of patients with cardiac conduction disorders and the left ventricle diastolic dysfunction. CONCLUSIONS: Nintedanib therapy was associated with stabilization of pulmonary function indicators, accompanied by a decrease in the dyspnea severity, increased tolerance for exercise, and decreased SSc activity. There was no significant effect of the drug on other clinical manifestations of SSc. CLINICAL IMPLICATIONS: Nintedanib can be used as a second-line therapy for the treatment of SSc-ILD with the ineffectiveness of immunosuppressive therapy. DISCLOSURES: No relevant relationships by Lidia Ananyeva No relevant relationships by Oxana Desinova No relevant relationships by Liudmila Garzanova No relevant relationships by Olga Koneva No relevant relationships by Olga Ovsyannikova No relevant relationships by Rushana Shayakhmetova No relevant relationships by Mayya Starovoytova" @default.
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• W4387247839 date "2023-10-01" @default.
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• W4387247839 title "THE STUDY OF NINTEDANIB EFFICACY IN PATIENTS WITH SYSTEMIC SCLEROSIS ASSOCIATED WITH INTERSTITIAL LUNG DISEASE" @default.
• W4387247839 doi "https://doi.org/10.1016/j.chest.2023.07.3550" @default.
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