Extract Interstitial lung disease (ILD) is a leading cause of morbidity and mortality in patients with systemic autoimmune rheumatic diseases (SARDs) [1]. In many cases, ILD may present as the first clinical manifestation of SARDs. The prevalence of ILD varies among SARDs, affecting up to 50% of patients with systemic sclerosis (SSc) and up to 15% of those with rheumatoid arthritis (RA) [2]. Unlike idiopathic pulmonary fibrosis (IPF), the most common form of ILD, which primarily affects older populations, RA-ILD and SSc-ILD impact more diverse demographics, often manifesting at a younger age with a less predictable disease course. Notably, shorter telomere length and older age are recognised risk factors for SARD-ILD [3 - 8]. Emerging therapies such as nintedanib (approved for SSc-ILD and progressive pulmonary fibrosis (PPF) in the USA and Europe) [9, 10], tocilizumab (approved for SSc-ILD in the USA) [11, 12], and nerandomilast (for PPF, not yet approved) [13] represent significant advancements in managing SARD-ILD. However, a deeper understanding of the molecular mechanisms driving ILD in SARD remains essential for developing more targeted therapies.
Extract Interstitial lung disease (ILD) is a leading cause of morbidity and mortality in patients with systemic autoimmune rheumatic diseases (SARDs) [1]. In many cases, ILD may present as the first clinical manifestation of SARDs. The prevalence of ILD varies among SARDs, affecting up to 50% of patients with systemic sclerosis (SSc) and up to 15% of those with rheumatoid arthritis (RA) [2]. Unlike idiopathic pulmonary fibrosis (IPF), the most common form of ILD, which primarily affects older populations, RA-ILD and SSc-ILD impact more diverse demographics, often manifesting at a younger age with a less predictable disease course. Notably, shorter telomere length and older age are recognised risk factors for SARD-ILD [3 - 8]. Emerging therapies such as nintedanib (approved for SSc-ILD and progressive pulmonary fibrosis (PPF) in the USA and Europe) [9, 10], tocilizumab (approved for SSc-ILD in the USA) [11, 12], and nerandomilast (for PPF, not yet approved) [13] represent significant advancements in managing SARD-ILD. However, a deeper understanding of the molecular mechanisms driving ILD in SARD remains essential for developing more targeted therapies.