Collagen vascular diseases in children

CVDs are a complex group of multisystem disorders that present diagnostic and therapeutic dilemmas in children. The past 30 years have seen extensive advances in defining CVD in children. Light has been shed on the cause and pathogenesis of immunologic disease. Common proinflammatory pathways that can contribute to disease expression include cytokine polymorphisms and MHC Class II linkage. Increasing awareness of pathogenesis has generated new markers of early disease that permit early intervention. Advances in immunosuppression and the ability to use a wide variety of steroid-sparing agents have made therapy of CVD less toxic and more efficacious. Sun avoidance and protection, physical therapy, psychosocial therapy, and nutritional evaluation are integral to the care of CVD. The future of childhood CVD lies in continuation of research into the immunologic basis of disease and integration of a multisystem therapeutic approach into standard practice.