Nintedanib and its combination with immunosuppressives in connective tissue disease-related interstitial lung diseases.

Background: Nintedanib reduces the decline of pulmonary function in patients with advancing lung fibrosis.

Aim: To assess the characteristics of the patients with connective tissue diseases (CTDs) related to interstitial lung disease (ILD) under nintedanib treatment.

Methods: The CTD-related ILD patients under nintedanib treatment who were followed up between 2020 and 2023 were included in the study. The clinical data of the patients before and after nintedanib were evaluated retrospectively.

Results: There were 36 patients (19 female) with a mean age of 65.2 ± 8.5 years who were treated with nintedanib. The median duration for nintedanib treatment was 19 months. The most common CTD was Sjogren's syndrome (36.1%), followed by systemic sclerosis (27.8%), rheumatoid arthritis (25%), undifferentiated CTD (8.3%), and inflammatory myositis (2.8%). Fifteen (41.7%) patients had impaired pulmonary function tests (FVC < 70 ml and/or DLco < 80 ml), and 23 (63.9%) patients had ≥ 20% involvement of parenchyma in high-resolution computed tomography (HRCT) before nintedanib. According to HRCT findings, 25 (69.4%) patients had the usual interstitial pneumonia pattern. All patients had 300 mg/day of nintedanib and received at least one immunosuppressive treatment during the study period. The mean %predicted value of FVC was 82.8 ± 17.6, and DLco was 65.3 ± 19.2 before nintedanib treatment. Following the 6-month follow-up, FVC showed an increase to 92.3 ± 15.8 (with an R correlation coefficient of 0.54, p = 0.025), and 22 (61.1%) patients exhibited either stabilization or regression of findings on HRCT.

Conclusions: Nintedanib emerges as a promising therapeutic agent compatible with immunosuppressives for treating progressive lung fibrosis in patients with CTD-related ILD.