Interstitial lung disease (ILD) frequently occurs in systemic sclerosis (SSc), significantly increasing the mortality risk. Diagnosing ILD associated with SSc (SSc-ILD) necessitates a vigilant approach due to the absence of respiratory symptoms in some patients. Early high-resolution computed tomography (HRCT) screening for SSc diagnosis is imperative, as ILD can manifest across all SSc patients. Regular monitoring, encompassing pulmonary function tests, symptom assessment and periodic HRCT scans, is crucial for monitoring disease progression and guiding treatment strategies. Immunotherapy, typically the primary treatment for SSc-ILD, along with nintedanib, an approved antifibrotic agent, has demonstrated effectiveness in preserving lung function. Effective diagnosis and management of SSc-ILD require a comprehensive patient-centred approach aimed at optimising outcomes and enhancing quality of life. © 2024 Andarini et al., published by Sciendo.