Systemic Sclerosis-Associated Interstitial Lung Disease: What We Know and How to Incorporate Guidelines Into Clinical Practice.

Abstract

Interstitial lung disease (ILD) affects 40% to 60% of patients with systemic sclerosis (SSc) and represents the leading cause of death. Although treatment options for SSc with ILD remain limited, randomized controlled trials have demonstrated the safety and efficacy of approved and unapproved therapies. However, numerous unanswered questions remain regarding management of SSc with ILD, and 3 recently published clinical practice guidelines highlight the complexity of treating this condition. These guidelines offer distinct and sometimes contrasting views on treatment of SSc with ILD, creating unique challenges for clinicians. The present review aimed to synthesize evidence regarding the management of SSc with ILD with a focus on how to apply the recent guidelines into practice, beginning with screening for disease and extending to the intricate decision-making surrounding treatment. Although an abundance of evidence supports screening all patients with SSc for ILD with high-resolution CT imaging of the chest at the time of diagnosis, emerging evidence underscores the importance of rescreening patients who possess ≥ 1 high-risk factors (male sex, Black race, advanced age, diffuse cutaneous disease, high modified Rodnan skin score, antitopoisomerase antibody positivity, nucleolar antinuclear antibodies, and impaired lung function) or if physiologic changes, new respiratory symptoms, or both attributable to ILD emerge. Patients with established SSc with ILD should be monitored closely for the development of progressive pulmonary fibrosis. The guidelines all recommended, with varying degrees of conditionality, treatment with mycophenolate, tocilizumab, rituximab, cyclophosphamide, and nintedanib. The quality of evidence was rated as low for most therapies, and the only therapy strongly recommended for SSc with ILD was mycophenolate. This review provides a comprehensive appraisal of current approaches to the diagnosis and treatment of SSc with ILD. It also highlights gaps in knowledge and proposes future opportunities to develop a precision-guided approach to diagnosing and treating SSc with ILD.