Kawasaki disease: Complications
- 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, also called mucocutaneous lymph node syndrome) is one of the most common vasculitides of childhood . KD also occurs rarely in adults. It is typically a self-limited condition, with fever and manifestations of acute inflammation lasting for an average of 12 days without therapy . However, complications such as coronary artery (CA) aneurysms, depressed myocardial contractility and heart failure, myocardial infarction, arrhythmias, and peripheral arterial occlusion may develop and lead to significant morbidity and mortality (table 1). Noncardiac complications may also affect children with KD.
The complications of KD are discussed in this topic review, with long-term cardiovascular sequelae of KD reviewed in greater detail separately. The epidemiology, etiology, clinical manifestations, diagnosis, and treatment of KD, refractory KD, and incomplete (atypical) KD are also presented separately. (See "Cardiovascular sequelae of Kawasaki disease" and "Kawasaki disease: Epidemiology and etiology" and "Kawasaki disease: Clinical features and diagnosis" and "Kawasaki disease: Initial treatment and prognosis" and "Refractory Kawasaki disease" and "Incomplete (atypical) Kawasaki disease".)
KD shock syndrome (KDSS), defined as sustained systolic hypotension (decrease in blood pressure greater than 20 percent from baseline) or clinical signs of poor perfusion, is a potentially life-threatening complication of KD [3,4]. It may be accompanied by multiple organ dysfunction syndrome (MODS), with MODS seen in 8 of 11 patients with KD and shock in one series . It is important to distinguish patients with KDSS from those with other causes of severe hypoperfusion since aggressive use of intravenous immune globulin (IVIG), which is essential for the successful control of KDSS, is not typically used in other types of shock. The presence of anemia and thrombocytosis is more common in KDSS as opposed to thrombocytopenia in toxic shock syndrome (TSS) and septic shock. The features that are most suggestive of KDSS, however, are younger age (mean age approximately three years in KDSS versus nine years for TSS)  and the presence of echocardiographic abnormalities, particularly valvulitis and coronary artery (CA) lesions. (See "Sepsis syndromes in adults: Epidemiology, definitions, clinical presentation, diagnosis, and prognosis", section on 'Multiple organ dysfunction syndrome' and "Initial evaluation of shock in children" and "Initial management of shock in children".)
In one case series from a single institution, 13 of 187 consecutive patients with KD (7 percent) presented with KDSS between 2003 and 2007 . All patients with KDSS received fluid resuscitation, and seven also received inotropic agents. Patients with shock syndrome were more likely than KD patients who were hemodynamically stable to have consumptive coagulopathy and cardiac abnormalities including impaired left ventricular systolic function, mitral regurgitation, and CA abnormalities. In addition, patients who presented with shock had higher C-reactive protein (CRP) levels than those with uncomplicated KD. Most importantly, children with KDSS were also less responsive to initial IVIG therapy and more commonly required additional treatment for their KD.
KD may cause both coronary artery (CA) dilatation as well as myocardial abnormalities. The most significant effects of KD involve pathologic changes in the coronary arteries. Acute, necrotizing arteritis, subacute and persistent arterial inflammation, and luminal myofibroblastic proliferation appear to affect most if not all children with KD . Depending on their severity, these processes may result in formation of aneurysms (defined as dilatation of coronary arteries to more than 4 mm internal diameter), ectasia (representing less severe yet still abnormal CA diameters), functional vasodilatory abnormalities despite a normal arterial diameter, and stenosis of the vascular lumen . (See "Cardiovascular sequelae of Kawasaki disease".)
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- CARDIAC COMPLICATIONS
- Coronary artery abnormalities
- - Risk factors for coronary artery aneurysms
- Depressed myocardial contractility
- MACROPHAGE ACTIVATION SYNDROME
- NONCORONARY VASCULAR INVOLVEMENT
- Urinary abnormalities and renal disease
- Gastrointestinal abnormalities
- Central nervous system
- INFORMATION FOR PATIENTS