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Critical care of the pediatric patient with rheumatic disease
Andrew I Shulman1, Marilynn Punaro
1Division 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.
Insights
Pediatric rheumatic diseases can be severe, requiring intensive care. Advances in therapy improve outcomes, but immunosuppressed patients with infections face risks, necessitating targeted treatments.
Area of Science:
- Pediatric Rheumatology
- Critical Care Medicine
- Immunology
Background:
- Childhood rheumatic diseases often require pediatric intensive care due to systemic illness and immunosuppressive treatments.
- Severe manifestations pose diagnostic and treatment challenges for pediatric rheumatologists and critical care physicians.
- Conditions like pediatric systemic lupus erythematosus and juvenile dermatomyositis can be life-threatening.
Purpose of the Study:
- To discuss complications of childhood rheumatic conditions requiring critical intervention.
- To highlight diagnostic and treatment challenges in severe pediatric rheumatic disorders.
- To explore the promise of targeted therapies based on disease pathogenesis.
Main Methods:
- Review of current literature on pediatric rheumatic diseases and critical care management.
- Analysis of severe disease manifestations and their impact on patient outcomes.
- Discussion of advances in understanding disease pathogenesis.
Main Results:
- Therapeutic advances have decreased severe complications and expanded treatment options.
- Patients with active rheumatic disease, secondary infections, and immunosuppression are at highest risk.
- Improved understanding of pathogenesis offers potential for more targeted therapies.
Conclusions:
- Childhood rheumatic conditions can necessitate intensive care interventions.
- Targeted therapies hold promise for managing these diseases more effectively with fewer adverse effects.
- Further research into disease mechanisms is crucial for developing advanced treatment strategies.
Purpose Of Review:
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.
Recent Findings:
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.
Summary:
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.
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