Abstract
Interstitial lung disease (ILD) affects 40-60% of patients with systemic sclerosis (SSc) and represents the leading cause of death. While treatment options for SSc-ILD remain limited, randomized controlled trials (RCTs) have demonstrated the safety and efficacy of approved and unapproved therapies. However, numerous unanswered questions remain regarding SSc-ILD management, and three recently published clinical practice guidelines highlight the complexity of treating this condition. These guidelines offer distinct and sometimes contrasting views on SSc-ILD treatment, creating unique challenges for clinicians. The present review aims to synthesize evidence regarding the management of SSc-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.
While an abundance of evidence supports screening all patients with SSc for ILD with a high-resolution computed tomography scan of the chest (HRCT) at the time of diagnosis, emerging evidence underscores the importance of re-screening patients who possess one or more high-risk factors (male sex, Black race, advanced age, diffuse cutaneous disease, high modified Rodnan skin score, anti-topoisomerase antibody positivity, nucleolar antinuclear antibodies, impaired lung function) or if physiological changes and/or new respiratory symptoms attributable to ILD emerge. Patients with established SSc-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-ILD was mycophenolate.
This review provides a comprehensive appraisal of current approaches to the diagnosis and treatment of SSc-ILD. It also highlights gaps in knowledge and proposes future opportunities to develop a precision-guided approach to diagnosing and treating SSc-ILD.