Kawasaki disease: Initial treatment and prognosis
- Robert Sundel, MD
Robert Sundel, MD
- Section Editor — Pediatric Rheumatology
- Associate Professor of Pediatrics
- Harvard Medical School
- Section Editors
- Marisa Klein-Gitelman, MD, MPH
Marisa Klein-Gitelman, MD, MPH
- Section Editor — Pediatric Rheumatology
- Chief, Division of Rheumatology
- Ann & Robert H. Lurie Children's Hospital of Chicago
- Professor of Pediatrics
- Northwestern University Feinberg School of Medicine
- Sheldon L Kaplan, MD
Sheldon L Kaplan, MD
- Editor-in-Chief — Pediatrics
- Section Editor — Pediatric Infectious Diseases
- Professor and Vice Chairman for Clinical Affairs
- Baylor College of Medicine
Kawasaki disease (KD), formerly called mucocutaneous lymph node syndrome, is one of the most common vasculitides of childhood . It is typically a self-limited condition with fever and manifestations of acute inflammation lasting for an average of 12 days without therapy. However, KD may cause cardiovascular complications, particularly coronary artery (CA) aneurysms. These, in turn, can lead to coronary occlusion and cardiac ischemia and result in significant morbidity or even mortality. (See "Cardiovascular sequelae of Kawasaki disease".)
The frequency of CA aneurysm development and associated morbidity and mortality have dramatically decreased as a result of intravenous immune globulin (IVIG) therapy. This therapy is effective for preventing CA abnormalities, but the benefits in children who have already developed CA aneurysms are more equivocal. Thus, expeditious diagnosis and timely treatment are critical to achieve the optimal clinical outcome. The initial treatment of KD is discussed in this topic review. The clinical manifestations, diagnosis, cardiovascular sequelae, and treatment of refractory KD are reviewed elsewhere. (See "Refractory Kawasaki disease" and "Kawasaki disease: Clinical features and diagnosis" and "Cardiovascular sequelae of Kawasaki disease".)
Theoretically, it should be possible to stratify therapy for KD according to disease severity defined by the likelihood of developing coronary artery (CA) aneurysms. Many risk scores have been proposed, but none are validated across different populations [2,3]. Since no criteria have been developed that can reliably distinguish children with no risk of developing severe disease at the time of initial presentation, all children diagnosed with KD or incomplete KD are treated at the time of diagnosis . Studies are ongoing to determine whether risk factors for failing to respond to intravenous immune globulin (IVIG) treatment might identify patients who would benefit from more aggressive initial therapy for KD. At this point, however, additional agents are routinely used only for children who fail to respond to standard therapy. (See "Refractory Kawasaki disease", section on 'Risk factors'.)
Guidelines by the American Heart Association (AHA) and the American Academy of Pediatrics (AAP) are available for the treatment of patients who fulfill the diagnostic criteria for KD (table 1) and for those who do not meet criteria, but are still at increased risk of developing coronary artery (CA) aneurysms (so-called incomplete KD) (algorithm 1) [4,5]. The recommended initial therapy includes intravenous immune globulin (IVIG; 2 g/kg) administered as a single infusion over 8 to 12 hours and aspirin (initial dose of 30 to 50 mg/kg daily divided into four doses). Patients are usually observed for 24 hours (minimum 12 hours) following completion of initial therapy to confirm resolution of fever. (See "Kawasaki disease: Clinical features and diagnosis" and "Incomplete (atypical) Kawasaki disease" and 'Refractory KD' below.)
A retrospective review evaluated 195 patients with KD who developed CA aneurysms at four centers in the United States from 1981 to 2006, 53 (27 percent) of whom were not treated for KD, because they did not fulfill diagnostic criteria. Application of the 2004 AHA/AAP guidelines would have resulted in the administration of IVIG therapy to all but three of these children (98 percent) .
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- INITIAL THERAPY
- Intravenous immune globulin
- - Efficacy and dosing
- - Administration
- - Timing of therapy
- - Type of intravenous immune globulin
- - Adverse effects
- ADJUVANT THERAPIES
- - Disease stratification
- Tumor necrosis factor inhibition
- Other therapies
- REFRACTORY KD
- Long-term morbidity
- Monitoring for fever
- Cardiac evaluations
- Physical activity
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS