Critical care of the pediatric patient with rheumatic disease

Abstract

Extensive systemic illness and treatment with immunosuppressive agents often require patients with rheumatic diseases to be monitored or managed in the pediatric intensive care unit. Additionally, severe disease-specific manifestations of childhood rheumatic disorders present pediatric rheumatologists and critical care physicians with diagnostic and treatment challenges. Although mortality from rheumatic disease in children is rare, the most severe diseases, such as pediatric systemic lupus erythematosus and juvenile dermatomyositis, remain life-threatening. Advances in therapy have reduced the incidence of severe complications of autoimmune and inflammatory diseases and have expanded treatment options. However, patients with active underlying rheumatic disease and secondary infection who are being treated with immunosuppressive agents are most at risk for poor outcomes. Here we discuss the complications of childhood rheumatic conditions that necessitate critical intervention. We discuss how improved understanding of the cellular and molecular basis of disease pathogenesis holds the promise of more targeted therapy without the adverse effects of global immunosuppression.