Evaluation of the gastrointestinal tract in juvenile systemic sclerosis: the paediatric gastroenterologist perspective.

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.