Evaluation of Severity in Juvenile Dermatomyositis and Adult-onset Dermatomyositis: a National Multicentric Retrospective Study

What this Trial Is About

Dermatomyositis (DM) is a rare autoimmune disease that causes muscle and skin inflammation and affects blood vessels. It can occur in both adults and children and may lead to serious complications like lung disease and infections, as well as muscle weakness. This research aims to compare the overall severity of DM at the time of diagnosis in juvenile patients versus adults, while also looking at differences in organ-specific severity, long-term damage, disease activity, and treatment-related complications between these groups. The study observes patients diagnosed with dermatomyositis, grouping them into juvenile-onset (under 18 years old) and adult-onset (18 years and older). It does not involve treatment but focuses on evaluating clinical severity using established classification criteria. Patients are assessed for severity in muscle, lung, digestive, and skin involvement at the start and followed over time to measure disease damage and activity, up to 10 years after diagnosis. Participants will undergo evaluations of their disease severity and organ involvement at diagnosis and during follow-up visits at 2, 5, and up to 10 years later. Researchers will collect data on remission status and ongoing disease activity, as well as complications from treatments. The main outcome is the number of patients with global disease severity at baseline, with additional measures for specific organ severity and damage scores to understand how the disease progresses and affects patients over time.

Evaluation of Severity in Juvenile and Adult-onset Dermatomyositis