The gastrointestinal (GI) tract is one of the most affected organs in systemic sclerosis (SSc), described in 42-78% of children with juvenile-onset SSc (jSSc). GI disease negatively impacts quality of life and increases disease-specific morbidity and mortality. Immune-mediated vascular, mucosal and neuromuscular compromise of the GI tract results in heterogeneous disease and variable clinical symptoms, severity, and disease progression. The burden of GI disease has a significant impact on clinical outcomes, and yet diagnosis and monitoring of GI disease remain challenging.The problem is multifactorial and includes symptoms that are non-specific and often overlooked, poor correlation between GI symptoms and testing, and limitations of GI-specific diagnostic testing. This review describes the clinical presentation and diagnostic work-up of upper and lower GI disease in jSSc via three distinct clinical scenarios, each encompassing a different segment of the GI tract (oropharynx/oesophagus, stomach/small bowel, colon/anorectum). Specifically, it highlights that the assessment of GI disease in jSSc warrants a high level of suspicion, timely evaluation, proper diagnostic testing, and most notably multidisciplinary collaboration.

Systemic sclerosis (SSc) is a rare, chronic connective tissue disease characterised by a complex and not yet fully understood pathophysiology, leading to a wide spectrum of clinical manifestations. Despite important advances over recent years in elucidating disease mechanisms, improving early recognition, and expanding therapeutic options, SSc continues to represent a major clinical challenge due to its heterogeneity and unpredictable course.Ongoing research efforts are essential to better define disease subsets, identify prognostic markers, and refine treatment strategies. In this context, this annual review provides a critical overview of the most relevant studies published in 2025, with a focus on advances in pathogenesis, organ involvement, therapeutic approaches, and patient outcomes in systemic sclerosis.