Introduction: Interstitial lung disease (ILD) is one of the most important manifestations of connective tissue diseases (CTD) due to its association with high morbidity and mortality.
Areas covered: Literature review focused on the evidence on efficacy and safety of biological therapy.
Expert opinion: Rituximab (RTX) is the most studied drug, though tocilizumab (TCZ) has methodologically more robust evidence, whereas abatacept (ABA) has only anecdotal reports. RTX studies suggest a clinically relevant effect on lung function and fibrosis in refractory to conventional treatment patients, with a good safety profile. Its multi-level efficacy in systemic sclerosis and the potentially more favorable response of anti-synthetase syndrome, especially when administered early in acute-onset or exacerbated ILD stand out over current standard of care, pending the availability of randomized controlled clinical trials. The significant and clinically meaningful benefit found in lung function and fibrosis with TCZ in faSScinate and focuSSced trials represents a change with respect to the usual practice, reinforcing the importance of treatment in early subclinical or clinical SSc-ILD patients with risk factors for ILD progression. This evidence has led to the inclusion of both RTX and TCZ in the expert-based therapeutic algorithms or recommendations for CTD-ILD management.
Keywords: Interstitial lung disease; anti-synthetase syndrome; biologic therapy; connective tissue diseases; efficacy; idiopathic inflammatory myopathies; rituximab; safety; systemic sclerosis; tocilizumab.