title: Critical care of the pediatric patient with rheumatic disease.
abstract: OBJECTIVE
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Rheumatology, Department of Pediatrics, University of Texas Southwestern Medical Center and Department of Rheumatology, Children's Medical Center, Texas Scottish Rite Hospital, Dallas, USA.
authors.forename: Andrew I
authors.initials: AI
authors.lastname: Shulman
authors.forename: Marilynn
authors.initials: M
authors.lastname: Punaro
mesh_terms: D002648:Child; D003422:Critical Care; D006402:Hematologic Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007239:Infections / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D015278:Intensive Care Units, Pediatric; D055501:Macrophage Activation Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D012216:Rheumatic Diseases / Q000150:complications* / Q000628:therapy